MitoDat database revised mtdat04081998.txt - S. Zullo MitoDat ID Number EC # Protein Name Organelle Compartment Pathways/Enzyme Complex Gene Symbol Gene Name GDB Accession Number MIM Accession Number PDD ID Number Cytogenetic Position Idiograms Mouse Gene Symbol Mouse Cytogenetic Position MGD Accession Number Mouse Link Drosophila Gene Symbol Drosophila Cytogenetic Position Fly Unique Identifier Maize Gene Symbol Maize cytogenetic Position Maize Accession Number Notes References MD100001 1.6.2.2 NADH2:cytochrome b5 oxidoreductase Outer membrane DIA1 diaphorase (NADH) (cytochrome b-5 reductase) G00-119-848 250800 22q13.31-qter hum_22 Dia1 15 MGD-LOC-7850 7854 rat liver Sottocasa67; Fedocheva85; Bernardi81 MD100005 1.99.1.5 Kynurenine 3-hydroxylase Outer membrane "rat liver, Neurospora" Schnaitman68; Okamoto69; Beattle68; Stevens59; Cassady71 MD100010 2.3.1.7 Acetyl-CoA:carnitine O-acetyltransferase Outer membrane CRAT carnitine acetyltransferase G00-359-759 600184 9q34.1 hum_09 rat heart Higgins70; Tyler92.page 262; Hashimoto82 MD100015 2.3.1.15 Acyl-CoA:L-glycerol-3-phosphate O-acyltransferase Outer membrane rat liver Stoffel68 MD100020 2.3.1.23 Acyl-CoA:lysolecithin O-acyltransferase Outer membrane LCAT lecithin-cholesterol acyltransferase G00-119-359 245900 16q22.1 hum_16 Lcat 8 MGD-LOC-10761 10774 rat liver Stoffel68 MD100021 2.3.1.- Acyl-CoA:lysophosphatidic acid O-acyltransferase Outer membrane rat liver Stoffel68 MD100025 2.7.1.1 ATP:D-hexose-6-phosphotransferase (types I & II) Outer membrane GCK "glucokinase (hexokinase 4, maturity onset diabetes of the young 2)" G00-127-550 138079 7p hum_01 "numerous, guinea pig intestine, liver, BRAIN" Kosow68; Hers51; Borreback70; Saltman53; Crane53; Srivastava68; Mayer71; Kropp70; Craven69 MD100030 3.1.1.4 Phosphate acyl-hydrolyase Outer membrane "rat liver, Torulopsis" Vignais69; Scherphof65; Rossi65; Waite67; Nachbaur69 MD100035 3.1.3.4 L-alpha-phosphatidate phosphohydrolase Outer membrane rat liver Stoffel68; Sedgwick65 MD100040 6.2.1.3 Acid:CoA ligase Outer membrane FACL1 "fatty-acid-Coenzyme A ligase, long-chain 1" G00-135-721 3q13 hum_03 rat liver Beaufay59; Farstad67; Pande68 MD100041 6.2.1.3 Acid:CoA ligase Outer membrane FACL2 "fatty-acid-Coenzyme A ligase, long-chain 2" G00-127-357 152425 4 hum_04 Facl2 UN MGD-LOC-10761 18754 none MD100045 Porin; Voltage-dependent anion-selective channel (VDAC) Outer membrane VDAC2 voltage-dependent anion channel 2 G00-138-281 Unassgined hexokinase-binding&glycerol kinase-binding protein de pinto87; Mannella84; Fiek82 MD100050 1.4.3.4 Monoamine oxidase A Outer membrane MAOA monoamine oxidase A G00-120-164 309850 Xp11.4-p11.3 hum_X Maoa X MGD-LOC-11115 11128 "marker enzyme, aka Monoamine oxidase aka Monoamine:O2 oxidoreductase (deaminating) aka amine oxidase A (B) aka tyramine oxidase aka tyraminase aka adrenalin oxidase. flavoprotein, reacts directly with molecular oxygen, an ancillary oxidation rxn of mito " Tyler92.page 98; Tyler92.page 174 MD100051 1.4.3.4 Monoamine oxidase B Outer membrane MAOB monoamine oxidase B G00-119-377 309860 Xp11.4-p11.3 hum_X Maob X MGD-LOC-11116 11129 "aka Monoamine oxidase aka Monoamine:O2 oxidoreductase (deaminating) aka amine oxidase A (B) aka tyramine oxidase aka tyraminase aka adrenalin oxidase. flavoprotein, reacts directly with molecular oxygen, an ancillary oxidation rxn of mito " none MD100055 anterograde receptors Outer membrane axons Schroer88 MD100065 retrograde receptors Outer membrane axons Schroer88 MD100075 Cytochrome b5 Outer membrane CYB5 cytochrom b-5 G00-125-236 250790 18q22.3-q23 hum_18 rat liver (also in ER) Ito80; Tyler92.page 174; Tyler92.page 170; Tyler92.table 4.7 MD100080 NADH-cytochrome b5 reductase Outer membrane DIA1 diaphorase (NADH) (cytochrome b-5 reductase) G00-119-848 250800 22q13.31-qter hum_22 rat liver Tyler92.page 170 MD100085 OM cytochrome b Outer membrane MTCYB cytochrome b G00-118-906 MTH14747-15887 mt-Cytb MT MGD-LOC-18340 18386 rat liver (also in ER) Ito80; Tyler92.page 170; Tyler92.table 4.7 MD100090 Long-chain fatty acyl-CoA synthetase Outer membrane CPS1 "carbamoyl-phosphate synthetase 1, mitochondrial" G00-119-799 237300 2q33-q36 hum_02 rat liver Tyler92.page 170 MD100091 Long-chain fatty acyl-CoA synthetase Outer membrane CAD "carbamoyl-phosphate synthetase 2, aspartate transcarbamylase, and dihydroorotase" G00-119-742 114010 2p22-p21 hum_02 none MD100095 2.3.1.21 "Carnitine O-Palmitoyltransferase (CPT I) (external, overt)" Outer membrane CPT1 carnitine palmitoyltransferase 1 G00-127-272 255120 1p32 hum_01 Cpt1 UN MGD-LOC-1122 1121 rat liver Woeltje90; Ruzicka77; Tyler92.page 210; Tyler92.page 205 MD100100 2.3.1.15 Glycerol 3-phosphate O-acyltransferase (GPAT) Outer membrane GPD2 glycerol-3-phosphate dehydrogenase 2 (mitochondrial) G00-354-558 Unassigned rat liver Tyler92.page 205 MD100101 2.3.1.15 Glycerol-phosphate acyltransferases Outer membrane rat liver Tyler92.page 170 MD100105 CDP-diacylglycerol cytidyltransferase Outer membrane rat liver Tyler92.page 170 MD100110 3.1.1.32 A1 Phospholipase Outer membrane rat liver Tyler92.page 170; Zurini81 MD100115 3.1.1.4 A2 Phospholipase Outer membrane none MD100120 3.1.1.5 B Phospholipase Outer membrane none MD100125 3.1.4.3 C Phospholipase Outer membrane none MD100130 3.1.4.4 D Phospholipase Outer membrane none MD100135 Receptors for binding to cytosol Outer membrane rat liver Tyler92.page 170 MD100140 Fatty acid elongation enzymes Outer membrane Colli69 MD100145 1.4.3.4 Amine oxidase (flavin-containing) Outer membrane ABP1 amiloride binding protein 1 (amine oxidase (copper-containing)) G00-127-105 104610 7q33-qter hum_01 "rat liver, aka Monoamine oxidase aka Monoamine:O2 oxidoreductase (deaminating) aka amine oxidase A (B) aka tyramine oxidase aka tyraminase aka adrenalin oxidase. flavoprotein, reacts directly with molecular oxygen, an ancillary oxidation rxn of mito " Noronha60; Sato84; Hawkins52; de Duve60; Aile59; Tyler92.page 191; Tyler92.page 255; Tyler92.page 174 MD100150 receptors for leader peptides Outer membrane Tyler92.page 174 MD100160 """Peripheral-type"" benzodiazepine receptors" Outer membrane BZRP benzodiazepine receptor (peripheral) G00-127-347 109610 22q13.31-qter hum_22 Porin? Anholt86; Tyler92.page 175 MD100165 2.4.1.1 Glucosyltransferase Outer membrane PTGB "phosphorylase, glycogen; brain" G00-120-326 138550 20q11.2 hum_20 rat liver Tyle92.page 183 MD100170 2.4.1.31 alpha-D-glucosyltransferase Outer membrane none MD100175 Mannosyltransferase Outer membrane rat liver Tyle92.page 183 MD100177 2.4.1.1.132 Mannosyltransferase II Outer membrane none MD100185 MOM19 Outer membrane "19 kDal,from Neurospora, receptors for precursors" Pfanner90 MD100190 MOM72 Outer membrane "72 kDal,from Neurospora, receptors for precursors" Pfanner90 MD100195 "GIP, general insertion protein" Outer membrane Mr~40 kDal Tyler92.page 460 MD100200 4.4.1.17 Cytochrome c-heme lyase Outer membrane Stuart90; Tyler92.page 461 MD100205 2.1.1.59 Cytochrome c-lysine methyltransferase Outer membrane none MD100210 1.9.3.1 Cytochrome C-oxidase Outer membrane none MD100225 2.3.1.15 glycerol-3-phosphate acyltransferase Outer membrane none MD100230 2.3.1.51 1-acylglycerol-3-phosphate acyltransferase Outer membrane none MD100235 2.7.7.41 CTP-phosphatidate cytidyiyltransferase Outer membrane none MD100290 Phosphatidyl-glycerolphosphate synthase Outer membrane none MD100291 3.1.3.27 Phosphatidylglycerol phosphatase Outer membrane aka Phosphatidyl-glycerolphosphate phosphatase aka Phosphatidylglycerophosphate phosphohydrolase none MD100295 Diphosphatidyl-glycerol synthase Outer membrane none MD200004 2.7.4.3 ATP:AMP phosphotransferase (iso)(& intracristal) Intermembrane space AMPK-PEN AMP-activated protein kinase 1p31 hum_01 various Brdiczka68; Schnaitman68; Novikoff52; Heldt67; Nielsen69 MD200005 2.7.4.3 Adenylate kinase (isoenzyme III) Intermembrane space AK1 adenylate kinase 1 G00-119-664 103000 9q34.1 hum_09 Ak1 2 MGD-LOC-248 245 "rat liver, marker enzyme" Tyler92.page 170 MD200006 Adenylate kinase (isoenzyme III) Intermembrane space AK2 adenylate kinase 2 G00-118-987 103020 1p34 hum_01 Ak2 4 MGD-LOC-249 246 none MD200007 2.7.4.3 Adenylate kinase (isoenzyme III) Intermembrane space AK3 adenylate kinase 3 G00-118-988 103030 9pter-p13 hum_09 Ak3 4 MGD-LOC-250 247 rat liver Tyler92.page 156 MD200007 2.7.4.3 Adenylate kinase III Intermembrane space AK3 adenylkinase 3 9pter-p13 hum_09 rat liver Tyler92.page 156 MD200010 2.7.4.6 ATP:nucleoside diphosphate phosphotransferase Intermembrane space NME2 "non-metastatic cells 2, protein expressed in" G00-128-120 156491 17q21-q22 hum_17 Nme2 UN MGD-LOC-11906 11924 rat liver Heldt67 MD200010 2.7.4.6 Nucleoside diphosphate kinase Intermembrane space NME2 "non-metastatic cells 2, protein expressed in" G00-128-020 156491 17q21-q22 hum_17 rat liver Tyler92.page 170 MD200015 4.4.1.17 Cytochrome c-heme lyase (membrane-bound) Intermembrane space rat liver Nicholson89; Tyler92.page 170 MD200020 Cytochrome c1-heme lyase (membrane-bound) Intermembrane space addition of heme to apocytochrome c1 Nicholson89 MD200025 1.8.3.1 Sulfite oxidase (cytochrome c reacting group) Intermembrane space rat liver Tyler92.page 170 MD200030 1.8.99.1 Sulfite-cytochrome c reductase Intermembrane space rat liver Tyler92.page 170 MD200035 1.6.2.2 IS cytochrome b5 (aka molybdohemoprotein sulfite oxidase Intermembrane space rat liver Tyler92.page 170; Tyler92.page 173 MD200040 1.11.1.5 Cytochrome c peroxidase (cytochrome c reacting group) Intermembrane space YEAST Tyler92.page 170 MD200050 1.3.3.3 Coproporphyrinogen III oxidase Intermembrane space CPO "coproporphyrinogen oxidase (coproporphyria, harderoporphyria)" G00-119-070 1213000 9 hum_09 none MD200105 2.7.4.3 adenylate kinase III Intermembrane Space rat liver Tyler92.page 156 MD200110 2.7.4.3 Adenylate kinase Intermembrane space Tyler92.page 178 MD300001 1.1.1.30 D-3-Hydroxybutyrate:NAD+ oxidoreductase (aka D3HBDH) Inner membrane BDH "3-hyroxybutyrate dehydrogenase (heart, mitochondrial)" G00-135-718 Unassigned numerous Lehninger60; Beaufay59; Norum66; Nielsen69; Tyler92.page 80 MD300005 1.1.1.37 L-Malate:NAD oxidoreductase (iso) Inner membrane MDH2 "malate dehydrogenase 2, NAD (mitochondrial)" G00-120-171 154100 7cen-q22 hum_07 Mor1 5 MGD-LOC-11359 11373 mdh2 6 12414 numerous Roodyn62; Parsons66; Brdiczka68 MD300010 1.1.1.41 LS-Isocitrate:NAD oxidoreductase (decarboxylating) Inner membrane numerous Brdiczka68; Ernster56; Schnaitman68 MD300011 1.1.1.42 LS-Isocitrate:NADP oxidoreductase (decarboxylating) Inner membrane IDH2 "isocitrate dehydrogenase 2, mitochondrial" G00-119-326 147650 15q21-qter hum_15 Idh2 7 MGD-LOC-10188 10198 idh2 6 12339 numerous Schnaitman68; Henderson65 MD300015 1.1.99.5 Glycerol-3-phosphate dehydrogenase Inner membrane "rat liver, brain, muscle, Locusta muscle, yeast" Zebe59; Fiala67; Tipton68; Dawson69; Lee69; Klingenberg70 MD300020 1.3.99.1 Succinate:(acceptor) oxidoreductase (aka s. dehydrogenase) Inner membrane SDH succinate dehydrogenase 1 (1 of 2 polypeptides) G00-119-589 185470 1p36-p35.1 hum_01 aka Succinic dehydrogenase aka fumarate reductase aka fumarate dehydrogenase aka fumaric hydrogenase. numerous Siess83; Munn74 MD300025 1.3.99.1 Succinate:(acceptor) oxidoreductase (aka s. dehydrogenase) Inner membrane SDHFP-PEN "succinate dehydrogenase, flavoprotein subunit" G00-378-037 1p36-p35.1 hum_01 "rat liver, Locusta, Musca, muscle" Brosemer 65; Lang58; Strecker55; Johnson62; Sacktor55 MD300030 1.5.1.2 L-Proline:NAD(P) 5-oxidoreductase Inner membrane PYCR1 pyrroline-5-carboxylate reductase 1 G00-135-716 179035 17 hum_17 numerous Kaplan53; Spiro61a; Spiro61b MD300035 1.6.1.1 NADPH2:NAD oxidoreductase Inner membrane numerous Munn74 MD300040 1.9.3.1 Cytochrome c:O2 oxidoreductase Inner membrane "rat, rabbit liver" Schnaitman68; Siekevitz53; Leuthardt48; Leuthardt49; Muller50; Sottocasa67c MD300045 2.1.3.3 Carbomyl phosphate:L-ornithine carbomyl transferase Inner membrane OTC ornithine carbamoyltransferase G00-119-468 311250 Xp21.1 hum_X Otc X MGD-LOC-12063 12084 "pig heart, rat heart, pigeon muscle, sheep tissues" Schnaitman68; Fritz63; Chase65; Snoswell72; Higgins70 MD300050 2.3.1.7 Acetyl-CoA:carnitine O-acetyltransferase Inner membrane "calf, human liver, rat liver" Norum64; Bremer67; Yates66; Norum66; Tyler92.page 262; Hashimoto82 MD300055 2.3.1.-??? Palmityl-CoA:carnitine palmityltransferase Inner membrane numerous Munn74 MD300060 2.6.1.1 L-Aspartate:2-oxoglutarate aminotransferase (iso) Inner membrane GOT2 "glutamic-oxaloacetic transaminase 2, mitochondrial (aspartate aminotransferase 2)" G00-120-009 138150 16q13 hum_16 guinea pig BRAIIN Craven69 MD300065 2.7.1.1 ATP:D-hexose-6-phosphotransferase (types I & II) Inner membrane HK1 hexokinase 1 G00-120-044 142600 10q22 hum_10 Hk1 10 MGD-LOC-9740 9750 none MD300066 2.7.1.1 ATP:D-hexose-6-phosphotransferase (types I & II) Inner membrane HK2 hexokinase 2 G00-137-213 2p13.1 hum_02 rat liver Nordlie61; Schick69 MD300070 3.6.1.1 Pyrophosphate phosphohydrolase Inner membrane PP pyrophoasphatase (inorganic) G00-119-505 179030 10q11.1-q24 hum_10 Pyp 10 MGD-LOC-12708 12732 numerous Munn74 MD300075 4.1.3.7 Citrate oxaloacetate-lyase (CoA-acetylating) Inner membrane CS citrate synthase G00-119-082 118950 12p11-qter hum_12 Cs 10 MGD-LOC-1188 1188 numerous Brdiczka68; Fritz63; Shepherd69; Marco69 MD300080 4.2.1.17 L-3-Hydroxyacyl-CoA hydro-lyase Inner membrane EHHADH enoyl-Coenzyme A hydratase/3-hydroxyacyl Coenzyme A dehydrogenase G00-141-631 261515 3q26.3-q28 hum_03 rat liver Stoffel68; Schneider48; Kennedy48 MD300085 4.99.1.1 Protohaem ferrolyase Inner membrane FECH ferrochelatase (protoporphyria) G00-127-282 177000 18q21.1-q21.31 hum_18 "rat liver, S. cerevisiae" Jones68; McKay69; Riethmuller64 MD300090 6.4.1.3 Propionyl-CoA:CO2 ligase (ADP) Inner membrane PCCA "propionyl Coenzyme A carboxylase, alpha polypeptide" G00-119-473 232000 13q32 hum_13 Pcca 14 MGD-LOC-12176 12198 "guinea pig heart, ox, rat liver" Brdiczka68; Scholte69; Wit-Peeters70; Lane60 MD300095 6.4.1.3 Propionyl-CoA:CO2 ligase (ADP) Inner membrane PCCA "propionyl Coenzyme A carboxylase, beta polypeptide" G00-119-474 232050 3q21-q22 hum_03 none MD300100 cardiolipin Inner membrane "considered to be located exclusively in the inner membrane, precursor mito proteins have affinity for this" Barnard79; Tyler92.page 140; Ou88; Tyler92.155 MD300105 Thermogenin (H+ & Cl- conducting) Inner membrane "brown fat mitochondria, functions as dimer of 32 kDal subunits and one GTP-binding site" Nedergaard84; Dawkins65; Tyle92.page 335 MD300110 one protein aligned with om porin Inner membrane none MD300115 second protein aligned with om porin Inner membrane none MD300120 ATP-dependent protease Inner membrane LON-PEN "Lon, ATP-dependent protease (homolog of bacterial Lon)" G00-269-860 Unassigned c-face of inner membrane Rapoport82 MD300121 ATP-dependent protease Inner membrane P26S4-PEN "protease, 26S, ubiquitin/ATP-dependent, subunit 4, 51kDa" G00-269-860 Unassigned none MD300125 specific adenine nucleotide exchange carrier Inner membrane ANT1 adenine nucleotide translocator 1 (skeletal muscle) G00-119-680 103220 4q35 hum_04 Tyler92.page 140 MD300130 mercurial-sensitive Pi carrier Inner membrane Tyler92.page 140 MD300135 Respiratory chain cytochromes Inner membrane Tyler92.page 162 MD300145 1.9.3.1 Cytochrome c oxidase Inner membrane marker enzyme Tyler92.page 174 MD300150 Metabolite carrier proteins Inner membrane "rat liver, in many tissues TCA cycle intermediates shuffled by these enzymes between cytosol and matrix" Tyler92.page 170; Tyler92.page 235 MD300170 2.7.3.2 Creatine kinase; ATP:creatine phosphotransferase (iso)(& intracristal) Inner membrane CKMT1 "creatine kinase, mitochondrial 1 (ubiquitous)" G00-119-780 123290 15 hum_15 Ckmt1 2 MGD-LOC-15209 15241 "muscle, BRAIN; C-face; mammal brain, duck muscle" Bieber 86; Tyler92.page 208; Tyler92.page 170; Muller85; Jacobs64; Brown68; Swanson67; Sullivan68 MD300171 2.7.3.2 Creatine kinase; ATP:creatine phosphotransferase (iso)(& intracristal) Inner membrane CKMT2 "creatine kinase, mitochondrial 2, sarcomeric" G00-119-780 123295 5q13.3 hum_05 "sarcomeric mitochondria, 11 exons over 37kb" Richard93; Bieber86; Tyle92.page 208 MD300175 Cytochrome P450 monooxygenase Inner membrane CYP7 "cytochrome P450, subfamily VII (cholesterol 7 alpha-monooxygenase)" G00-132-221 118455 8q11-q12 hum_08 "adrenal, liver, kidney" Tyler92.page 170 MD300180 1.14.15.6 Cytochrome P450 Inner membrane POR P450 (cytochrome) oxidoreductase G00-120-309 124015 7q11.2 hum_07 Por 6 MGD-LOC-12589 12612 "adrenal cortex, testis, ovary, placenta,steroidogenic tissue (much [smaller] in liver and kidney)" none MD300185 3.1.1.4 Phospholipase A2 Inner membrane PLA2G2A "phospholipase A2, group IIA (platelets, synovial fluid)" G00-120-296 172411 1 hum_01 regulated by free [Ca+2] in vivo (ref. #61) Zurini81; Lenting68 MD300190 3.1.1.4 Phospholipase A2 Inner membrane PLA2G1B "phospholipase A2, group IB (pancreas)" G00-120-715 172410 12q23-qter hum_12 Pla2g1b UN MGD-LOC-17665 17709 none MD300195 3.1.1.4 Phospholipase A2 Inner membrane PLA2G4 "phospholipase A2, group IV" G00-134-687 1q24-q25 hum_01 none MD300200 3.1.1.4 Phospholipase A2 Inner membrane PLA2G5 "phospholipase A2, group V" G00-307-456 1q24-q25 hum_01 none MD300205 1.6.1.1 Transhydrogenase Inner membrane bovine heart Tyler92.table 4.6 MD300210 Fatty acid elongation enzymes Inner membrane Colli69 MD300230 1.1.99.1 Choline dehydrogenase Inner membrane Tyler92.page 173 MD300235 Heme biosynthesis enzymes (some) Inner membrane Tyler92.page 173 MD300250 "ATP synthase, subunit 6" Inner membrane MTATP6 ATP synthase 6 G00-118-897 516060 MT8527-9207 mt-ATP6 MT MGD-LOC-15731 15573 Tyler92.page 175 MD300251 "ATP synthase, subunit 8" Inner membrane MTATP8 ATP synthase 8 G00-118-898 516070 MT8366-8572 mt-Atp8 MT MGD-LOC-15730 15572 "encoded in mtDNA, ATPase A6L, may be analogous to ATPase associated protein (aap I protein) of yeast" Tyler92.page 378 MD300255 Adenine nucleotide carrier Inner membrane ANT1 adenine nucleotide translocator 1 (skeletal muscle) G00-119-680 103220 4q35 hum_04 Ant1 RE MGD-LO9C-15250 15284 Tyler92.page 175 MD300256 Adenine nucleotide carrier Inner membrane ANT2 adenine nucleotide translocator 2 (fibroblast) G00-125-190 300150 Xq13-q26 hum_X none MD300257 Adenine nucleotide carrier Inner membrane ANT3 adenine nucleotide translocator 3 (liver) G00-125-184 300151 Xp22.32;Yp11.3 hum_X; hum_Y none MD300260 2.7.1.108 Dolichol Kinase Inner membrane rat liver Tyler92.page 183 MD300265 Fatty acyl-CoA lysophospholipid acyltransferase Inner membrane Tyler92.page 184 MD300270 fatty acyl-CoA synthetases Inner membrane Tyler92.page 184 MD300275 2.3.1.86 fatty acyl-CoA synthase Inner membrane none MD300280 Monocarboxylate carrier Inner membrane Tyler92.page 197 MD300285 Carnitine-acyl-carnitine exchange carrier Inner membrane CRAT carnitine acetyltransferase G00-359-759 600184 9q34.1 hum_09 transports activated fatty acids Ruzicka77; Tyler92.page 210; Tyler92.page 204; Tyler92.page 207 MD300290 2.3.1.21 "Carnitine palmitoyltransferase (CPT II) (internal, latent)" Inner membrane CPT1 carnitine palmitoyltransferase 1 G00-127-272 255120 1p32 hum_01 Woeltje90; Tyler92.page 207 MD300295 amino acid carriers Inner membrane Tyler92.page 220 MD300330 Glutamate carrier Inner membrane SLC1A1 "solute carrier family 1 (neuronal/epithelial high affinity glutamate transporter, system Xag), member 1" G00-316-130 133550 9p24 hum_09 Tyler92.page 247 MD300331 Glutamate carrier Inner membrane SLC1A2 "solute carrier family 1 (glial high affinity glutamate transporter), member 2" G00-371-479 600300 Unassigned none MD300332 Glutamate carrier Inner membrane SLC1A3 "solute carrier family 1 (glial high affinity glutamate transporter), member 3" G00-371-499 600111 5p13 hum_05 none MD300333 Glutamate carrier Inner membrane SLC1A3 "solute carrier family 1 (glutamate/neutral amino acid transporter), member 4" G00-371-499 600111 5p13 hum_05 none MD300340 Aspartate carrier Inner membrane Tyler92.page 247 MD300345 Malate carrier Inner membrane Tyler92.page 247 MD300350 Ornithin/citrulline exchange carrier Inner membrane none MD300355 Anion channel Inner membrane opens to initiate uncoupling to release heat Nicholls84; Tyler92.page 336 MD300360 1.5.99.8 Proline dehydrogenase Inner membrane "M face of inner membrane, FAD (to 1-proline-5-carboxylate, spontaneous to glutamate semialdehyde, substrate for below)" Tyler92.page 256 MD300362 1.5.1.1-2 obs. Inner membrane none MD300365 1.5.1.2 1-proline-5-carboxylate dehydrogenase Inner membrane "M face of inner membrane, NAD(+) to L-glutamate, aka L-proline:NAD(P)(+) 5-oxidoreductase" Tyler92.page 256 MD300370 NADH-UBIQUINONE Subfraction 1:iron-sulfur flavoprotein fraction (FP) Inner membrane three subunits in 1:1:1 ratio Tyler92.page 308 MD300375 1.6.5.3 NADH-UBIQUINONE Subfraction 2:iron-sulfur protein fraction (IP) Inner membrane UQCRFS1 "ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1" G00-217-069 191327 19q12 hum_19 at least 6 subunits Tyler92.page 308 MD300385 NADH-UBIQUINONE Subfraction 3:hydrophobic fraction (HP) Inner membrane "ca 15 subunits, phospholipids, and iron and labile sulfur, ca 70% of total complex protein" Tyler92.page 308 MD300400 1.6.99.5 (NADH-Q reductase) Inner membrane rotenone-sensitivity dependent on phospholipid Tyler92.page 306 MD300405 Mitoskelin Inner membrane Price89 MD300410 1.3.5.1 SUCCINATE-UBIQUINONE subunit CII-1 Inner membrane "70 kDal, flavoprotein containing FAD" Hederstedt85 MD300411 1.3.5.1 SUCCINATE-UBIQUINONE subunit CII-2 Inner membrane "27 kDal, iron-sulfur protein with one tetranuclear [4Fe4S]iron-sulfur center, S-3" Hederstedt85 MD300412 1.3.5.1 SUCCINATE-UBIQUINONE subunit CII-3 Inner membrane "15 kDal, function unclear, present in cytochrome b560 preps, probably with heme prosthetic group" Tyler92.page 310 MD300413 1.3.5.1 SUCCINATE-UBIQUINONE subunit CII-4 Inner membrane "13.5 kDal, function uncl., pres. in cytoc b560 preps, may be identical to QPs, prtn functions? as a specific ubiquinone apoprtn, req. for conv. of s.DH to s.reductase, and prob. resp. for binding and stabilizing ubisemiquinone radical" Tyler92.page 310 MD300415 (Succinate-Q reductase) Inner membrane none MD300420 1.3.99.1 Succinate dehydrogenase Inner membrane SDH succinate dehydrogenase 1 (1 of 2 polypeptides) G00-119-589 185470 1p36-p35.1 hum_01 flavin-linked Tyler92.page 362 MD300421 "Succinate dehydrogenase, flavoprotein subunit" Inner membrane SDHFP-PEN "succinate dehydrogenase, flavoprotein subunit" G00-378-037 Unassigned none MD300425 QH2-CYTOCHROME c Subunit I Inner membrane "45.5 kD, core protein I, close resemblance to chaperone proteins (!9:49)" Hatefi85; Weiss87; Parlo84 MD300426 QH2-CYTOCHROME c II Inner membrane "44.0 kD, core protein II" Hatefi85; Weiss87 MD300427 QH2-CYTOCHROME c III Inner membrane "42.5 kD, cytochrome b, (2 b hemes:c1), only mitochondrially-encoded subunit" Hatefi85; Weiss87 MD300428 QH2-CYTOCHROME c IV Inner membrane "31.0 kD, cytochrome c1" Hatefi85; Weiss87 MD300429 QH2-CYTOCHROME c V Inner membrane "24.6 kD, Iron-sulfur protein (FeSIII, 1:c1)" Hatefi85; Weiss87 MD300430 QH2-CYTOCHROME c VI Inner membrane "13.4 kD, (?) QPc" Hatefi85; Weiss87 MD300431 QH2-CYTOCHROME c VII Inner membrane 11.0 kD Hatefi85; Weiss87 MD300432 QH2-CYTOCHROME c VIII Inner membrane "9.2 kD, Hinge protein" Hatefi85; Weiss87 MD300433 QH2-CYTOCHROME c IX Inner membrane 4.8 kD Hatefi85; Weiss87 MD300434 QH2-CYTOCHROME c X Inner membrane 3.0 kD Hatefi85; Weiss87 MD300435 QH2-CYTOCHROME c XI Inner membrane 6.4 kD Hatefi85; Weiss87 MD300441 1.9.3.1 Cytochrome c oxidase Subunit I Inner membrane MTCO1 cytochrome c oxidase I G00-118-900 MTH5904-7444 mt-Co1 MT MGD-LOC-18343 18389 mt-Co1 MT Fbgn0013674 "#1; mitochondrially encoded, 514 aa; 56,993 D, binding to redox groups to form the catalytic subunits" Azzi90 MD300442 1.9.3.1 Cytochrome c oxidase II Inner membrane MTCO2 cytochrome c oxidase II G00118-901 MTH7586-8262 mt-Co2 MT MGD-LOC-18342 18388 mt-Co2 MT Fbgn0013675 "#3; mitochondrially encoded, 227 aa; 26,049 D, binding to redox groups to form the catalytic subunits" Azzi90 MD300443 1.9.3.1 Cytochrome c oxidase III Inner membrane MTCO3 cytochrome c oxidase III G00-118-902 MTH9207-9990 mt-Co3 MT MGD-LOC-18341 18387 mt-Co3 MT Fbgn0013676 "#2; mitochondrially encoded, 269 aa; 29,918 D, (?) H+-translocation" Azzi90 MD300444 1.9.3.1 Cytochrome c oxidase IV Inner membrane COX4 cytochrome c oxidase subunit IV G00-119-068 123864 16q24.1 hum_16 Cox4 8 MGD-LOC-1101 1100 "#4; 147 aa; 17,153 D, (?) regulation of catalytic activity and spatial organization of catalytic subunits in the membrane" Azzi90 MD300445 1.9.3.1 Cytochrome c oxidase Va/Vb Inner membrane "#5; 105 aa; 12,434 D, (?) regulation of catalytic activity and spatial organization of catalytic subunits in the membrane " Azzi90 MD300446 1.9.3.1 Cytochrome c oxidase Vb/Via/a Inner membrane COX5B cytochrome c oxidase subunit Vb G00-127-530 123866 2cen-q13 hum_02 Cox5b UN MGD-LOC-1103 1102 "#6; 98 aa; 10,670 D, (?) regulation of catalytic activity and spatial organization of catalytic subunits in the membrane" Azzi90 MD300447 1.9.3.1 Cytochrome c oxidase VIb/ Inner membrane COX6B cytochrome c oxidase subunit VIb G00-128-117 124089 19q13.1 hum_19 "#7; 85 aa; 10,068 D, (?) regulation of catalytic activity and spatial organization of catalytic subunits in the membrane" Azzi90 MD300448 1.9.3.1 Cytochrome c oxidase VII/c Inner membrane COX7C cytochrome c oxidase subunit VIIc G00-128-569 Unassigned Azzi90 MD300449 1.9.3.1 Cytochrome c oxidase VIa/VIb/b Inner membrane "#8; occur in tissue-specific isoforms; 84 aa; 9,418 D, (?) regulation of catalytic activity and spatial organization of catalytic subunits in the membrane" Azzi90 MD300450 1.9.3.1 Cytochrome c oxidase VIa Inner membrane COX6A2 cytochrome c oxidase subunit VIa polypeptide 2 G00-136-189 Unassigned none MD300451 1.9.3.1 Cytochrome c oxidase VIa Inner membrane COX6A1 cytochrome c oxidase subunit VIa polypeptide 1 G00-118-774 Unassigned none MD300452 1.9.3.1 Cytochrome c oxidase VIc Inner membrane COX6C cytochrome c oxidase subunit VIc G00-118-775 124090 Unassigned "#9; 73 aa; 8,480 D, (?) regulation of catalytic activity and spatial organization of catalytic subunits in the membrane" Azzi90 MD300453 1.9.3.1 Cytochrome c oxidase VIIb Inner membrane COX7B cytochrome c oxidase subunit VIIb G00-138-315 Unassigned "#10; 56 aa; 6,350 D, (?) regulation of catalytic activity and spatial organization of catalytic subunits in the membrane" Azzi90 MD300454 1.9.3.1 Cytochrome c oxidase VIIa/VIIIc Inner membrane "#11; occur in tissue-specific isoforms; 56 aa; 6,243 D, (?) regulation of catalytic activity and spatial organization of catalytic subunits in the membrane" Azzi90 MD300455 1.9.3.1 Cytochrome c oxidase VIIa Inner membrane COX7A1 cytochrome c oxidase subunit VIIa polypeptide 1 (muscle) G00-118-776 123995 19q13.1 hum_19 none MD300456 1.9.3.1 Cytochrome c oxidase VIIa Inner membrane COX7A2 cytochrome c oxidase subunit VIIa polypeptide 2 (liver) G00-135-979 123996 4q31-q35 hum_04 none MD300457 1.9.3.1 Cytochrome c oxidase VIIa Inner membrane COX7A3 cytochrome c oxidase subunit VIIa polypeptide 3 (liver) G00-135-980 123997 14 hum_14 none MD300458 1.9.3.1 Cytochrome c oxidase VIIc/VIIa Inner membrane "#12; 47 aa; 5,541 D, (?) regulation of catalytic activity and spatial organization of catalytic subunits in the membrane" Azzi90 MD300459 1.9.3.1 Cytochrome c oxidase VIII/VIIIb Inner membrane "#13; occur in tissue-specific isoforms; 46 aa; 4,962 D, (?) regulation of catalytic activity and spatial organization of catalytic subunits in the membrane" Azzi90 MD300460 1.9.3.1 Cytochrome c oxidase VIII Inner membrane COX8 cytochrome c oxidase subunit VIII G00-119-796 123870 11q12-q13 hum_11 none MD300465 F1-FO-ATPase alpha Inner membrane ATP5A1 "ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit, isoform 1, cardiac muscle" G00-137-185 9 and 18 hum_09; hum_18 Atp5a1 UN MGD-LOC-472 470 "54 Kd, alpha-beta pairs are catalytic centers, nucleotide binding sites have regulatory (a) and catalytic (beta) function, close resemblance to chaperone proteins(!9:49)" Parlo84; Tyler92.page 380 MD300466 3.6.1.34 F1-FO-ATPase alpha Inner membrane ATP5A2 "ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit, isoform 2, non-cardiac muscle" G00-137-186 9 and 18 hum_09;hum_18 none MD300467 3.6.1.34 F1-FO-ATPase beta Inner membrane ATP5B "ATP synthase, H+ transporting, mitochondrial F1 complex, beta" G00-119-718 102910 12p13-qter hum_12 50 Kd Tyler92.page 380 MD300468 3.6.1.34 F1-FO-ATPase gamma Inner membrane ATP5C1 "ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 1" G00-137-187 Unassigned "33 Kd, proton gate between Fo and catalytic centers; stabilizes a3b3 structure" Tyler92.page 380 MD300469 3.6.1.34 F1-FO-ATPase gamma Inner membrane ATP5C2 "ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 2" G00-217-058 Unassigned none MD300470 3.6.1.34 F1-FO-ATPase delta Inner membrane ATP5D "ATP synthase, H+ transporting, mitochondrial F1 complex, delta subunit" G00-132-360 Unassigned "17 Kd; with epsilon, forms binding btwn OSCP and F6 of Fo and F1ATPase" Tyler92.page 380 MD300471 3.6.1.34 F1-FO-ATPase epsilon Inner membrane ATP5E "ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit" G00-137-118 Unassigned 5.7 Kd Tyler92.page 380 MD300472 3.6.1.34 Subunit b Inner membrane ATP5F1 "ATP synthase, H+ transporting, mitochondrial F0 complex, subunit b, isoform 1" G00-137-189 Unassigned homologous to bacterial b and chloroplast I Tyle92.page 378 MD300473 DCCD-binding proteolipid Inner membrane "in oligomeric form, f(x) as H+ conducting channel across im lipid bilayer; homologous to bacterial c, and chloroplast III, encoded by yeast mtDNA" Tyler92.page 378; Tyler92.page 85 MD300474 3.6.1.34 OSCP (Oligomycin sensitivity conferring protein) Inner membrane ATP5I "ATP synthase, H+ transporting, mitochondrial F0 complex, subunit e (? oligomycin sensitivity conferring protein)" G00-137-192 Unassigned "18 Kd, homologous to bacterial delta" Maclennan68; Tyler92.page 378; Tyler92.page 380 MD300475 Coupling factor 6 (F6) Inner membrane 8 Kd Tyler92.page 378; Tyler92.page 380 MD300476 Coupling factor B (FB) Inner membrane "14.6 Kd, (?) organizes and regulates proton channel, inhibits nonspecific proton leakage; no ATP synthase gene appears to exist for this subunit:!7:36 " Walker87; Tyler92.page 378; Tyler92.page 380 MD300477 3.6.1.34 Subunit d Inner membrane ATP5H "ATP synthase, H+ transporting, mitochondrial F0 complex, subunit d" G00-137-191 Unassigned Tyler92.page 378 MD300478 inhibitor protein Inner membrane "9.6 Kd, regulates ""backflow"" of energy from ATP; to F1-ATPase" Pullman63; Tyler92.page 366; Hansford76; Ebel75 MD300480 Type I NADH dehydrogenase Inner membrane "isolated from membranes tx with phospholipase, contains no phospholipid nor Q" Tyler92.page 306 MD300485 1.6.99.3 NADH dehydrogenase Inner membrane none MD300490 1.6.99.5 NADH dehydrogenase (quinone) Inner membrane none MD300495 1.8.3.1 Sulfite oxidase Inner membrane "liver, interacts with complex III cytochrome c" Tyler92.page 317 MD300500 Nicotinamide Nucleotide Transhydrogenase Inner membrane "97-120 kDal, single polypeptide, catalytic center accessible to substrates in matrix space:NADP+ + NADH + H+ ---> NADPH + H+ + NAD+" Tyler92.page 347f MD300505 2.7.7.1 Nicotinamide Nucleotide adenylyltransferase Inner membrane White80; Mandel90; Tyler92.page 388ff MD300510 1.18.1.2 Adrenodoxin reductase Inner membrane NADP-specific FAD-containing flavoprotein; 54kDal; weakly bound to membrane Omura66 MD300515 Adrenodoxin Inner membrane iron-sulfur proetin containing 2 Fe and 2 labile sulfur; 12.5 kDal; readily detached thus functions as a peripheral protein Omura66 MD300520 Cytochrome P450 Inner membrane "ca 60 kDal, functions as terminal oxidase reacting with molecular oxygen and as substrate-binding site during hydroxylation rxns; integral protein" Omura66 MD300525 1.14.15.6 Cytochrome P450scc Inner membrane "specific P450 to convert cholesterol to pregnenolone by cleaving the side chain, strongly dependent on binding of single cardiolipin to lipid-effector binding site on enzyme" Tyler92.page 341 MD300530 22-OHase Inner membrane cholesterol to 22-hydroxycholesterol none MD300535 20-OHase Inner membrane "22-hydroxycholesterol to 20, 22-dihydroxycholesterol" none MD300540 C20-C22 lyase Inner membrane "20, 22-dihydroxycholesterol to pregnenolone" none MD300545 Cytochrome P45011beta Inner membrane distinct from P450scc none MD300550 11beta-hydroxylase Inner membrane "11-deoxycortisol to cortisol or 11-deoxycorticosterone(from ER) to corticosterone, in adrenal cortex, thought to also catalyze 18-hydroxylase rxn" none MD300555 1.1.1.- 18-hydroxysteroid dehydrogenase Inner membrane 18-hydroxycorticosterone to aldosterone none MD300560 1alpha-hydroxylase Inner membrane 25-hydroxyvitamin D3 to 1alpha-25-dihydroxyvitamin D3 (most potent known effector of increased intestinal absorption of Ca+2 and mobilization of bone mineral Mandel90; Tyler92.page 343f MD300565 1.14.15.3 1-hydroxylase Inner membrane none MD300570 P450D1alpha Inner membrane none MD300575 Mersalyl-sensitive Pi carrier Inner membrane Tyler92.figure 8.1 MD300580 Malate-Pi antiport carrier Inner membrane Tyler92.figure 8.1 MD300585 Citrate-Malate antiport carrier Inner membrane Tyler92.figure 8.1 MD300590 Dicarboxylate carrier Inner membrane Tyler92.page 407 MD300595 Tricarboxylate carrier Inner membrane enriching mito membranes with cholesterol increases rate of citrate export on this (!10:49) Parlo84; Tyler92.page 407 MD300600 ATP-Mg-Pi exchange carrier Inner membrane liver Aprille88 MD300605 NAD+ carrier Inner membrane plants Neuberger85 MD300610 CoASH carrier Inner membrane plants Neuberger84 MD300615 Branched-chain 2-oxo acid carrier Inner membrane "heart muscle, may be identical with pyruvate carrier" Hutson90 MD300620 4.1.1.65 Phosphatidylserine decarboxylase Inner membrane "on c face of inner membrane, forms phosphatidyl ethanolamine, biogenesis system" none MD300625 4'PP-adenyltransferase Inner membrane Tahiliani87 MD300630 2.7.1.24 Dephospho-CoA kinase Inner membrane Tahiliani87 MD300635 Dihydroorotate-ubiquinone reductase Inner membrane c-face of inner membrane Miller69 MD300640 1.3.1.14 (s) Dihydroorotate oxidoreductase (NAD+) Inner membrane none MD300641 1.3.1.15 (s) Dihydroorotate oxidoreductase (NADP+) Inner membrane none MD300645 2.3.1.37 5-aminolevulinate synthase (ALA synthase) Inner membrane ALAS1 "aminolevulinate, delta-, synthase 1" G00-120-543 125290 3p21.1-p12 hum_03 first and rate-determining step from succinylCoA and glycine. Final three steps by enzymes associated with the inner membrane Tyler92.page 470 MD300650 Coproporphyrinogen III oxidase Inner membrane intermembrane space of liver mitos none MD300660 1.3.3.3 Coproporphyrinogen oxidase Inner membrane none MD300665 1.3.3.4 Protoporphyrinogen oxidase Inner membrane PPOX protoporphyrinogen oxidase G00-118-852 Unassigned integral with regions exposed at both C and M face none MD300670 4.99.1.1 Ferrochelatase Inner membrane integral with regions exposed at both C and M face none MD300675 Cytochrome c-heme lyase (membrane-bound) Inner membrane "activity in innermembrane space, rat liver" Nicholson89; Tyle92.page 170 MD300780 Cytochrome c1-heme lyase (membrane-bound) Inner membrane "activity in innermembrane space, addition of heme to apocytochrome c1" Nicholson89 MD300785 1.3.99.1 Succinate dehydrogenase Inner membrane rat liver Sato84 MD300801 1.6.5.3 NADH Dehydrogenase 1 Inner membrane MTND1 NADH dehydrogenase 1 G00-118-911 MTH3307-4262 mt-Nd1 MT MGD-LOC-17597 17641 none MD300802 1.6.5.3 NADH Dehydrogenase 2 Inner membrane MTND2 NADH dehydrogenase 2 G00-118-912 516001 MTH4470-5511 mt-Nd2 MT MGD-LOC-18339 18385 none MD300803 1.6.5.3 NADH Dehydrogenase 3 Inner membrane MTND3 NADH Dehydrogenase 3 G00-118-913 MTH10059-10404 mt-Nd3 MT MGD-LOC-18338 18384 none MD300804 1.6.5.3 NADH Dehydrogenase 4 Inner membrane MTND4 NADH Dehydrogenase 4 G00-118-914 MTH10760-12137 mt-Nd4 MT MGD-LOC-18337 18383 none MD300805 1.6.5.3 NADH Dehydrogenase 4L Inner membrane MTND4L NADH Dehydrogenase 4L G00-118-915 MTH10470-10766 mt-Nd4l MT MGD-LOC-18336 18382 none MD300806 1.6.5.3 NADH Dehydrogenase 5 Inner membrane MTND5 NADH Dehydrogenase 5 G00-118-916 MTH12337-14148 mt-Nd5 MT MGD-LOC-18335 18381 none MD300807 1.6.5.3 NADH Dehydrogenase 6 Inner membrane MTND6 NADH Dehydrogenase 6 G00-118-917 MTL14673-14149 mt-Nd6 MT MGD-LOC-18334 18380 none MD400001 1.1.1.35 L-3-Hydroxyacyl-CoA:NAD oxidoreductase Matrix rat liver Brdiczka68 MD400005 1.1.1.37 L-Malate:NAD+ oxidoreductase (iso) (aka m. dehydrogenase) Matrix "numerous, marker enzyme, some bound to M face of im" Roodyn62; Parsons66; Brdiczka68; Tyler92.page 166 MD400010 1.1.1.41 LS-Isocitrate:NAD oxidoreductase (decarboxylating) Matrix numerous Brdiczka68; Ernster56; Schnaitman68 MD400011 1.1.1.42 LS-Isocitrate:NADP oxidoreductase (decarboxylating) Matrix numerous Schnaitman68; Henderson65 MD400015 1.5.1.12 1-Pyrroline-5-carboxylate:NAD(+) oxidoreductase Matrix rat liver Brunner69 MD400020 1.4.1.3 L-Glutamate NAD(P) oxidoreductase (deaminating) Matrix numerous Munn74 MD400025 2.1.3.3 Carbomyl phosphate:L-ornithine carbomyl transferase Matrix "rat, rabbit liver" Schnaitman68; Siekevitz53;Liuthardt48; Leuthardt49; Muller50; Sottocasa67C MD400030 2.3.1.7 Acetyl-CoA:carnitine O-acetyltransferase Matrix "pig heart, rat heart, pigeon muscle, sheep tissues" Schnaitman68; Fritz63; Chase65; Snoswell72; Higgins70; Tyler92.page 262; Tyler92.page 209; Hashimoto82 MD400035 2.6.1.1 L-Aspartate:2-oxoglutarate aminotransferase (iso) Matrix numerous Munn74 MD400040 2.7.4.6 ATP:nucleoside diphosphate phosphotransferase Matrix rat liver Heldt67 MD400045 4.1.1.9 Malonyl-CoA decarboxylase Matrix Scholte69 MD400050 4.1.3.7 Citrate oxaloacetate-lyase (CoA-acetylating) Matrix numerous Brdiczka68; Zebe60; Shepherd69; Marco69 MD400055 6.2.1.1 Acetate:CoA ligase (AMP) Matrix "rat liver, ruminants" Snoswell72; Cook69; Aas68 MD400056 6.2.1.2 Acid:CoA ligase (AMP) Matrix "ox, rat liver" Aas68 MD400058 6.2.1.4 Succinate:CoA ligase (GDP) Matrix "pig heart, rat liver" Cha64; Heldt67 MD400060 6.4.1.1 Pyruvate:CO2 ligase (ADP) Matrix numerous Brdiczka68; Ballard70; Keech63; Gull69; Bottger69 MD400065 6.4.1.3 Propionyl-CoA:CO2 ligase (ADP) Matrix "guinea pig heart, ox, rat liver" Brdiczka68; Scholte69; Wit-Peeters70; Lane60 MD400070 Actin Matrix Etoh90 MD400075 Myosin Matrix Fakekas87 MD400080 6.3.5.5 Carbamoyl phosphate synthetase I Matrix liver Tyler92.page 74 MD400085 2.6.1.1 Aspartate aminotransferase Matrix Tyler92.page 74; Tyler92.page 191 MD400090 1.4.1.2 glutamate dehydrogenase Matrix "liver and kidney especially NAD or NADP, (plants, & microbes, often NAD- or NADP-specific)" Tyler92.page 219 MD400095 3.1.- calcium-dependent phospholipase Matrix Tyler92.page 121 MD400100 2.6.1.1 alanine aminotransferase Matrix Tyler92.page 137 MD400105 1.4.1.2 ornithine aminotransferase Matrix Tyler92.page 137 MD400110 2.3.1.37 5-aminolevulinate synthase Matrix Tyler92.page 137 MD400115 1.15.1.1 Superoxide dismutase (Mn form) (SOD) Matrix "isoenzyme of cytoplasm form is from different ancestral gene, only known example, but homologous to prokaryotic SOD" Tyler92.page 140; Tyler92.page 178; Tyler92.page 49 MD400120 1.1.1.41 Isocitrate dehydrogenase Matrix some bound to M face of im; Mn2+ Tyler92.page 166; Tyler92.page 226 MD400125 ATP-dependent protease Matrix LON-PEN "Lon, ATP-dependent protease (homolog of bacterial Lon)" Unassigned human hippocampus cDNA library Wang93 MD400126 ATP-dependent protease Matrix P26S4-PEN "protease, 26S, ubiquitin/ATP-dependent, subunit 4, 51kDa" Unassigned none MD400130 2.7.4.6 Nucleosidediphosphate kinase Matrix Tyler92.page 175; Tyler92.page 235 MD400135 GTP-AMP phosphotransferase Matrix Tyler92.page 175 MD400140 1.1.99.5 Glycerol-3-phosphate dehydrogenase Matrix Tyler92.page 178 MD400145 GTP-specific succinyl-CoA synthetase Matrix "extrahepatic cells(?), functions during ketone body formation (!Section 5.2.5) and in heme biosynthesis (!Section 9.5.1)" Jenkins88 MD400150 "1.1.1.38, 1.1.1.40" Malic enzyme [aka Malate dehydrogenase (oxaloacetate-decarboxylating)] Matrix "malate to pyruvate, mito in intestinal, brain, and tumor cells where leads to enhanced unregulated cholesterol synthesis, but in most vert. tissues is cytosolic" Moreadith84; Tyler92.page 243 MD400155 3.5.1.2 Glutaminase Matrix "aka L-Glutamine amidohydrolase (iso). glutamine to glutamate and ammonia, intestine, tumor cells, rat liver." Brandon67; Mazelis57; Tyler92.page 243; Tyler92.page 245 MD400160 1.11.1.6 Catalase Matrix "mainly in peroxisomes, but also at least in rat heart" Nohl80 MD400163 1.11.1.9 Glutathione peroxidase Matrix "rat liver mainly in cytosol, but ca 15% activity in matrix; attack peroxidized fa's released from membrane by phospholipase A2" Flohe79 MD400170 1.2.4.2 2-oxoglutarate dehydrogenase (decarboxylating) Matrix E1 Lybuarev89 MD400175 2.3.1.61 Dihydrolipoamide succinyltransferase Matrix E2 Lybuarev89 MD400180 1.8.1.4 Dihydrolipoamide dehydrogenase Matrix "E3, possibly identical to pyruvate DH subunit; only deficiency in TCA cycle enzymes, infantile encephalmyopathy" Lybuarev89 MD400185 4.2.1.3 Aconitase Matrix "isoenzyme in cytosol, aka aconitate hydratase, Citrate (isocitrate) hydro-lyase" Stoffel64; Stoffel65; Lyubarev89; Beinert86; Tyler92.page 224; Tyler92.page 227 MD400190 1.1.1.42 "Isocitrate dehydrogenase, NAD(P)-linked " Matrix NADP-linked also here and in cytosol Lyubarev89; Dalziel80; Huang84; Sies82; Tyler92.page 224; Tyler92.page 178 MD400195 2.7.4.6 Nucleosidediphosphate kinase Matrix Lyubarev89 MD400200 Mitochondrial L-Malate dehydrogenase Matrix "heart muscle, 66-70 kDal, with 2 subunits, reversible and equilibrium strongly in favor of malate, but rapid removal of oxaloacetate by condensation with acetylCoA to form citrate shifts equilibrium" Lyubarev89; Tyler92.page 71; Tyler92.page 224 MD400205 Citrate synthase { (re) 4.1.3.28 (si) 4.1.3.7 } Matrix "formerly citrate condensing enzyme, heart form has 2 identical 100kDal subunits" Lyubarev89; Tyler92.page 71; Tyler92.page 226 MD400210 2.6.1.19 GABA-2-OG aminotransferase Matrix "formation and release of GABA by presynaptic cells, uptake and metabolism of GABA by glial cells" Bradford77; Tyler92.page 242 MD400215 1.2.1.24 Succinate semialdehyde dehydrogenase (NAD+) Matrix "In Brain, up to 1/3 of 2-oxoglutarate formed by IDH is removed to glutamate, then to cytosol, udergoing decarboxylation to GABA, some GABA is taken into mito and metabolized to succinate" Bradford77; Tyler92.page 242 MD400220 1.2.1.16 Succinate semialdehyde dehydrogenase (NADP+) Matrix none MD400225 6.2.1.4 "Succinyl-CoA Synthetase, GDP-forming" Matrix "alpha subunit, 33.5 kDal, site of phosphorylation" Collier78 MD400226 6.2.1.5 "Succinyl-CoA Synthetase, ADP-forming" Matrix "aka Succinate:CoA ligase (ADP). Heart muscle, 80 kDal, catalytic center at interface of two subunits. artichoke tuber." Sano61; Collier78 MD400227 Succinyl-CoA Synthetase Matrix "aka succinate thiokinase, " Lyubarev89; Tyler92.page 71 MD400230 2.6.1.1 mitochondrial Aspartate aminotransferase Matrix Lyubarev89; Tyler92.page 72 MD400235 Anchor proteins Matrix "isoenzyme in cytoplasm from same gene, mt isoenzyme not acetylated at N terminus" Tyler92.page 178 MD400240 1.3.99.1 Succinate dehydrogenase Matrix "ferriflavoprotein, covalently bound FAD prosthetic group, tightly bound to inner membrane, catalytic center facing matrix space, part of complex II (succinate-Q reductase)" Tyler92.page 232 MD400245 4.2.1.2 Fumarase Matrix "aka fumarate hydratase, akaL-Malate hydro-lyase. heart muscle form with 4 identical subunits combine 194 kDal, a few cases of deficiency" Suzuki89; Lyubarev89; Koide65; Tyler92.page 71; Tyler92.page 178; Tyler92.page 233 MD400250 1.6.1.1 Transhydrogenase Matrix "transfers reducing equivalents between NADPH to NAD+, metabolizing TCA, involved at isocitrateDH" Rydstrom76;Tyler92.page 230 MD400260 6.2.1.1 Acetyl-CoA synthetase Matrix Tyler92.page 205 MD400265 6.2.1.17 Propionyl-CoA synthetase Matrix liver Groot76; Tyler92.page 205 MD400270 4.1.1.41 Propionyl-CoA carboxylase Matrix "liver, biotin containing" Tyler92.page 206 MD400275 5.1.99.1 Methylmalonyl-CoA racemase Matrix liver Tyler92.page 206 MD400280 5.4.99.2 Methylmalonyl-CoA mutase Matrix "aka 2-Methylmalonyl-CoA CoA-carbonylmutase, liver, one of the few requiring 5'-deoxyadenosylcobalamin, from vitamin B12" Graham69; Tyler92.page 206 MD400285 6.4.1.1 Pyruvate carboxylase Matrix "exclusively matrix space, acts with PEPCK to convert P to PEP in liver and kidney " Tyler92.page 235 MD400290 6.2.1.2 Butyryl-CoA synthetase Matrix Tyler92.page 205 MD400295 Short-chain fatty acyl-CoA Dehydrogenase Matrix Ikeda83; Tyler92.page 210 MD400296 6.2.1.2 Medium-chain fatty acyl-CoA dehydrogenase Matrix Ikeda83 MD400297 6.2.1.3 Long-chain fatty acyl-CoA dehydrogenase Matrix Ikeda83 MD400300 4.2.1.17 Enoyl-CoA hydratase Matrix Tyler92.page 211 MD400305 1.1.1.35 beta-Hydroxyacyl-CoA dehydrogenase Matrix aka 3-hydroyacyl-CoA dehydrogenase Tyler92.page 71; Tyler92.page 178 MD400310 2.3.1.16 AcetylCoA acyltransferase (thiolysis) Matrix 261510 "aka Acyl-CoA:acetyl-CoA acyltransferase aka ACETYL-COA C-ACYLTRANSFERASE aka BETA-KETOTHIOLASE aka 3- KETOACYL-COA THIOLASE. mito and peroxisome have specific forms. Disease: PSEUDO-ZELLWEGER SYNDROME. Locusta flight muscle, rat heart, pigeon muscle" Rowsell63; Fellman69; Tyler92.page 71; Tyler92.page 178; Tyler92.page 241 MD400315 4.1.1.1 Branched-chain 2-oxo acid dehydrogenase complex Matrix "aka 2-Oxo-acid carboxy-lyase, rat kidney, irreversible, similar to PDH complex" Brandon67; Mazelis57; Ballard69; Yeaman86; Ikeda83; Tyler92.page 221 MD400320 4.1.1.- Phosphoenolpyruvate carboxykinase (matrix PEPCK) Matrix "cytosolic and matrix space (species & tissue differences in !5:38) to undergo net oxidation, beta-ox intermediates converted to acetylCoA thru this enzyme" Soling82; Tyler92.page 222; Tyler92.page 235; Newsholme83 MD400325 short-chain fatty acyl-CoA synthetase Matrix rat liver Tyler92.page 170 MD400330 1.3.1.34 "2,4-dienoyl-CoA reductase (NADPH)" Matrix NADP-linked Tyler92.page 214 MD400335 5.3.3.8 "cis-3,trans-2-enoyl-CoA isomerase" Matrix beta-oxidation of unsaturated fatty acids DCI dodecanoyl-coenzyme A delta-isomerase 16p13.3 hum_16 Janssen94 MD400340 2.3.1.9 Acetyl-CoA acetyltransferase Matrix "liver, extrahepatic cells" Tyler92.page 215; Tyler92.page 217 MD400344 4.1.3.4 Hydroxymethylglutaryl-CoA lyase Matrix aka 3-Hydroxy-3-methylglutaryl-CoA acetoacetate lyase. rat liver de Duve55; Kuff54; Blaschko55; Mahler58; Fenwick58; Shepherd55; Tyler92.page 216 MD400345 4.1.3.5 Hydroxymethylglutaryl-CoA synthase Matrix First enzyme of ketogenesis 1p12-p13 hum_01 "aka Hydroxymethylglutaryl-CoA reductase, aka 3-Hydroxy-3-methylglutaryl-CoA acetoacetyl-CoA-lyase (CoA acetylating). rate determining enzyme of cholesterol synthesis, in tumors increased cholesterol content, enzyme loses cholesterol feedback. rat liver." Boukaftane94; Mahler58; Shepherd55; Shepherd54; Kickman54 MD400350 Fatty acyl-CoA hydrolase Matrix liver Tyler92.page 216 MD400355 1.1.1.30 NAD-linked D-3-hydroxybutyrate dehydrogenase Matrix nonruminant liver (cytosolic in ruminants) Tyler92.page 216 MD400360 1.1.1.30 D-3-hydroxybutyrate dehydrogenase Matrix extrahepatic cells Tyler92.page 217 MD400365 2.8.3.5 3-oxoacid CoA-transferase Matrix "pig heart, extrahepatic cells, aka succinyl-CoA-actoacetate transferase, aka Succinyl-CoA:3-oxo-acid CoA-transferase" Clements70; Tyler92.page 217 MD400370 Aminotransferases Matrix Rat liver Tyler92.page 170 MD400375 1.4.1.2 Glutamate dehydrogenase Matrix "Rat liver, brain ammonia toxicity probably due to its effect on this enzyme's reaction equilibrium" Tyler92.page 170 MD400376 1.4.1.3 "Glutamate dehydrogenase, NAD(P)+" Matrix Tyler92.page 170 MD400377 1.4.1.4 "Glutamate dehydrogenase, NADP+" Matrix Tyler92.page 170 MD400380 6.3.5.5 Carbamoylphosphate synthetase I (urea cycle enzyme) Matrix Rat liver Tyler92.page 170 MD400385 2.1.3.3 Ornithine transcarbamylase (urea cycle enzyme) Matrix Rat liver Tyler92.page 170 MD400390 2.6.1.2 Alanine aminotransferase Matrix "aka L-Alanine:2-oxoglutarate aminotransferase, aka ALANINE AMINOTRANSFERASE aka GLUTAMIC--PYRUVIC TRANSAMINASE aka GLUTAMIC--ALANINE TRANSAMINASE. rat liver, pig heart, intestines, oxidation of gluamate to alanine" van Kempen65; Salganicoff65; Waksman68; Sato84; Tyler92.page 243 MD400395 6.3.4.16 Carbamoyl phosphate synthetase I (ammonia) Matrix "apparently rate limiting step in urea synthesis, activity dependent on ammonia, ornithine, and N-acetylglutamate, allosteric activator, in elasmobranchs is gln-dependent (CPSIII), in amphibs & mammals depends on ammonia (CPSI), in ureotelic animals" Tyler92.page 245 MD400400 6.3.1.2 Glutamine synthetase Matrix "analogous to CPE in uricotelic animals eg., birds and some reptiles, glutaminerather than citrulline is transported out and converted to uric acid" Tyler92.page 246 MD400405 3.5.3.1 Arginase Matrix "releases urea; in elasmobranchs is mitochondrial, in amphibs and mammals is cytosolic" Mommsen89; Tyler92.page 245; Mori87 MD400410 2.1.3.3 Ornithine transcarbamylase Matrix Tyler92.page 247; Mori87 MD400415 4.2.1.1 Carbonic anhydrase Matrix "320kDal, two similar subunits, Requires MgATP and free Mg+2,15-25% of total matrix-space protein in liver of ureotelic animals, apparently absent from heart, brain and kidney but present in liver and skeletal muscle" Tyler92.page 246; Dodgson80 MD400420 1.2.1.41 a glutamate-glutamate gamma-semialdehyde dehydrogenase Matrix to replenish ornithine along with next enzyme in liver Tyler92.page 249 MD400425 a glutamate gamma-semialdehyde aminotransferase Matrix replenishes ornithine Tyler92.page 249 MD400430 N-acetylglutamate synthase Matrix "uses acetyl-CoA and glutamate, allosterically activated by arginine" Sonoda83; Tyler92.page 251 MD400435 "1.2.2.2,1.2.4.1" Pyruvate dehydrogenase (decarboxylating)=E1 Matrix "bovine heart, (2 subunits, 41 kD, 36 kD" Wieland83; Tyler92.page 197 MD400440 2.3.1.12 Dihydrolipoamide acetyltransferase=E2 Matrix "bovine heart, 52kD single chain" Wieland83; Tyler92.page 197 MD400445 1.8.1.4 Dihydrolipoamide dehydrogenase=E3 Matrix "bovine heart, 110 kD dimer of 2 55kD monomers" Wieland83; Tyler92.page 197 MD400450 2.7.1.99 Pyruvate dehydrogenase kinase Matrix "bovine heart, (2 subunits, 48, 45 kD)" Wieland83; Tyler92.page 198 MD400455 3.1.3.43 Pyruvate dehydrogenase phosphate phosphatase Matrix "bovine heart, (2 subunits, 97, 50 kD)" Wieland83; Tyler92.page 198 MD400460 Carnitine-palmitoyl-CoA transferase Matrix Is it mito or cyto??? Tyler92.page 481 MD400465 2.3.1.21 "Carnitine palmitoyltransferase (CPT II) (internal, latent)" Matrix CPT1 carnitine palmitoyltransferase 1 1p32 hum_01 none MD400470 1.1.99.5 glycerol 3-phosphate dehydrogenase Matrix "flavoprotein, mammalian tissue and insect flight muscle" Tyler92.page 255 MD400475 1.1.99.1 Choline dehydrogenase Matrix "choline to betaine aldehyde, linked directly to respiratory chain" Tyler92.page 256f MD400480 1.2.99.3 (acceptor) Aldehyde dehydrogenase I (uM Km) Matrix "most mammalian livers, low Km(uM), principle enzyme oxidizing acetaldehyde during ethanol oxidation, betaine aldehyde to betaine, a 1 carbon methyl or hydroxymethyl donor, linked directly to respiratory chain" Sato84; Tyler92.page 256f MD400481 1.2.99.3 Aldehyde dehydrogenase II (mM Km) Matrix "most mammalian livers & human brain, high Km(mM), glutamic-gamma-semialdehyde dehydrogenase in human liver, succinate-semialdehydehyde dehydrogenase in brain " Sato84; Tyler92.page 256f MD400485 1.2.1.3 "Aldehyde dehydrogenase, NAD+" Matrix none MD400486 1.2.1.4 "Aldehyde dehydrogenase, NADP+" Matrix none MD400487 1.2.1.5 "Aldehyde dehydrogenase, NAD(P)+" Matrix none MD400490 1.5.99.1 Sarcosine dehydrogenase Matrix "sarcosine to glycine, covalently bound FAD and tightly cound folate" none MD400491 1.5.99.2 Dimethylglycine dehydrogenase Matrix "rat liver, dimethylglycine to sarcosine, covalently bound FAD and tightly bound folate" Cook83; Tyler92.page 256; Tyler92.page 207 MD400495 "1.4.4.2, 2.1.2.10" glycine synthase Matrix "aka Glycine-cleavage enzyme. liver form has 4 protein components in multienzyme complex similar to 2-oxo acid dehydrogenase, forms CO2, NH4+, methylene tetrahydrofolate, NADH, H+ form glycine" Tyler92.page 257 MD400500 2.1.2.2 Serine hydroxymethyltransferase Matrix "aka serine hydroxymethylase, isozyme in cytosol, glycine+methylene tetrahydrofolate to serine and tetrahydrofolate" Tyler92.page 257 MD400510 2.6.1.4 glycine aminotransferase Matrix Tyler92.page 424 MD400515 2.7.7.7 DNA polymerase gamma Matrix mtDNA replication POLG DNA polymerase gamma 174763 15q24-q26 hum_15 Polg 7E McDaniel96; Ropp96; Tyler92.page 484; Walker97; Zullo97 MD400520 Exonuclease G Matrix none MD400525 Maturase enzymes Matrix "known largely from yeast mito intron , Intron 2 COB maturase, Intron 4 COB m." Tyler92.page 451; Tyler92.page 455 MD400530 6.1.1.- amino acyl-tRNA synthetases Matrix Attardi90 MD400535 nuclear RNA component required for processing Matrix Chang87 MD400540 Ribosomal protein S-5 Matrix "var 1 protein in yeast, Absent in N. crassa ""poky"" mutant, encoded by mtDNA " Tyler92.page 189 MD400545 "Ribosomal proteins, as many as 85 different proteins" Matrix Tyler92.page 455 MD400640 1.3.1.- 2-methyl branched chain enoyl-CoA reductase Matrix "anaerobic, in Ascaris?, heme synthesis" Komuniecki89; Tyler92.page 399 MD400645 2.3.1.37 5-aminolevulinate synthase Matrix "functions in formation of porphyrin nucleus, heme synthesis" Tyler92.page 424 MD400650 1.2.1.- N-acetylglutamate synthetase Matrix regulated by arginine transport into mitos Freeland84 MD400655 Mitochondrial Processing Peptidase (MPP) Matrix "ca 50 kDal, 26% aa identity with PEP, protein family includes subunits I and II of complex III" Weiss90; Schneider90 MD400660 Processing-enhancing protein (PEP) Matrix "ca 50 kDal, 26% aa identity with MPP, protein family includes subunits I and II of complex III" Weiss90; Schneider90 MD400665 Hsp60 Matrix matrix space analogue of cytosolic Hsp70 Ellis90 MD400670 Dihydroorotate-ubiquinone reductase Matrix "on C-face of inner membrane, involved in pyrimidine syntehsis " Miller69 MD400675 NAD-linked Dehydrogenase Matrix succinate to succinate semialdehyde Tyler92.page 491 MD400680 NADP-linked reductase Matrix "succinate semialdehyde to 4-hydroxybutyrate, in 4-Hb acidemia" Rating84; Tyler92.page 491 MD400685 4'PP-adenyltransferase Matrix Tahiliani87 MD400690 2.7.1.24 Dephospho-CoAkinase Matrix Tahiliani87 MD400695 2.8.1.1 rhodanese aka Thiosulfate sulfurtransferase Matrix Thiosulphate:cyanide sulphurtransferase "aka thiosulfate-cyanide sulfurtransferase. widely distributed in mammalian tissues, forms sulfite and thiocyanate, Fe-S center synthesis. serum albumin-sulfane complex prob. prim. cyanide disposal system. most vital site of cyanide action is COX of CNS" Cerletti86; Beaufay64; Baudhuin69; Brouwe73 MD400700 2.3.1.37 5-aminolevulinate synthase (ALA synthase) Matrix first and rate-determining step from succinylCoA and glycine. Final three steps by enzymes associated with the inner membrane Tyler92.page 470 MD400705 1.14.11.8 6N-trimethyllysine-2-oxoglutarate dioxygenase Matrix Tyler92.page 482 MD400710 1.4.1.2 Glutamate dehydrogenase Matrix liver Tyler92.page 152 MD400715 1.3.99.1 Succinate dehydrogenase Matrix rat liver Sato84 MD400720 4.1.3.7 citrate synthase Matrix aka CITRATE (SI)-SYNTHASE aka CONDENSING ENZYME aka CITRATE CONDENSING ENZYME aka CITROGENASE aka OXALOACETATE Tyler92.page 152 MD400901 Matrix Mitochondrial DNA Replication/ transcription MT7SDNA 7S DNA G00-118-896 MTH5721-16104 none MD400902 Inner membrane Attachment site of mitochondrial genome to inner membrane MTATT Membrane Attachment Site G00-118-899 MTH15925-499 none MD400903 Matrix Conserved Sequence Block I in D-loop region MTCSB1 Conserved Sequence Block I G00-118-903 MTL235-213 none MD400904 Matrix Conserved Sequence Block II in D-loop region MTCSB2 Conserved Sequence Block II G00-118-904 MTL315-299 none MD400905 Matrix Conserved Sequence Block III in D-loop region MTCSB3 Conserved Sequence Block III G00-118-905 MTL363-346 none MD400906 Matrix Mitochondrial genome replication MTHPR Replication Primer G00-118-907 MTL321-317 none MD400907 Matrix Mitochondrial H-strand transcription/replication MTHSP1 Major H Strand Promoter G00-118-908 MTH545-567 none MD400908 Matrix Mitochondrial H-strand transcription/replication MTHSP2 Minor H Strand Promoter G00-118-909 MTH645-645 none MD400909 Matrix Mitochondrial L-strand transcription/replication MTLSP L Strand Promoter G00-118-910 MTL445-392 none MD400910 Matrix Mitochondrial L-strand origin of replication MTOLR Light strand origin of replication G00-118-919 MTL5805-5729 mt-Olr MT MGD-LOC-18333 18379 none MD400911 Matrix Mitochondrial protein synthesis/mitochondrial ribosomes MTRNR1 12S rRNA G00-118-920 MTH648-1601 mt-Rnr1 MT MGD-LOC-18332 18378 none MD400912 Matrix Mitochondrial protein synthesis/mitochondrial ribosomes MTRNR2 16S rRNA G00-118-921 MTH1671-3229 mt-Rnr2 MT MGD-LOC-18331 18377 none MD400913 Matrix MTRNR3 5S-like sequence G00-118-922 MTL3229-3206 none MD400914 Matrix Mitochondrial protein synthesis MTTA tRNA alanine G00-118-923 MTL5655-5587 none MD400915 Matrix mitochondrial transcription MTTAS termination associated sequence G00-118-924 MTL16172-16157 none MD400916 Matrix Mitochondrial protein synthesis MTTD tRNA aspartic acid G00-118-925 MTH7518-7585 none MD400917 Matrix Mitochondrial protein synthesis MTTE tRNA glutamic acid G00-118-926 MTL14742-14149 none MD400918 Matrix Mitochondrial genome transcription MTTER Transcription terminator G00-118-927 MTH3237-3249 none MD400919 Matrix Mitochondrial protein synthesis MTTF tRNA phenylalanine G00-118-928 MTH577-647 none MD400920 Matrix Mitochondrial genome transcription MTTFH mitochondrial transcription factor binding site H G00-118-929 MTH523-550 none MD400921 Matrix Mitochondrial genome transcription MTTFL mitochondrial transcription factor binding site L G00-118-930 MTL445-418 none MD400922 Matrix Mitochondrial genome transcription MTTFX mitochondrial transcription factor binding site X G00-118-931 MTL260-233 none MD400923 Matrix Mitochondrial genome transcription MTTFY mitochondrial transcription factor binding site Y G00-118-932 MTL303-276 none MD400924 Matrix Mitochondrial protein synthesis MTTG tRNA glycine G00-118-933 MTH9991-10058 none MD400925 Matrix Mitochondrial protein synthesis MTTH tRNA histidine G00-118-934 MTH12138-12206 none MD400926 Matrix Mitochondrial protein synthesis MTTI tRNA isoleucine G00-118-935 590045 MTH4263-4331 none MD400927 Matrix Mitochondrial protein synthesis MTTK tRNA lysine G00-118-936 MTH8295-8364 none MD400928 Matrix Mitochondrial protein synthesis MTTL1 tRNA leucine 1 (UUA/G) G00-118-937 590050 MTH3230-3304 none MD400929 Matrix Mitochondrial protein synthesis MTTL2 tRNA leucine 2 (CUN) G00-118-938 MTH12266-12336 none MD400930 Matrix Mitochondrial protein synthesis MTTM tRNA methionine G00-118-939 MTH4402-4469 none MD400931 Matrix Mitochondrial protein synthesis MTTN tRNA asparagine G00-118-940 MTL5729-5657 none MD400932 Matrix Mitochondrial protein synthesis MTTP tRNA proline G00-118-941 MTL16023-15955 none MD400933 Matrix Mitochondrial protein synthesis MTTQ tRNA glutamine G00-118-942 MTL4400-4329 none MD400934 Matrix Mitochondrial protein synthesis MTTR tRNA arginine G00-118-943 590005 MTH10405-10469 none MD400935 Matrix Mitochondrial protein synthesis MTTS1 tRNA serine 1 (UCN) G00-118-944 MTL7516-7445 none MD400936 Matrix Mitochondrial protein synthesis MTTS2 tRNA serine 2 (AGU/C) G00-118-945 MTH12207-12265 none MD400937 Matrix Mitochondrial protein synthesis MTTT tRNA threonine G00-118-946 MTH15888-15953 none MD400938 Matrix Mitochondrial protein synthesis MTTV tRNA valine G00-118-947 590105 MTH1602-1670 none MD400939 Matrix Mitochondrial protein synthesis MTTW tRNA tryptophan G00-118-948 MTH5512-5576 none MD400940 Matrix Mitochondrial protein synthesis MTTY tRNA tyrosine G00-118-949 MTL5891-5826 none MD400941 Matrix Mitochondrial protein synthesis MTTC tRNA cysteine G00-138-133 MTL5826-5761 none MD400942 Matrix Mitochondrial genome replication MTOHR Heavy Strand origin of replication G00-118-918 MTH110-441 none MD400943 Matrix Mitochondrial genome transcription MTTFH Mitochondrial Transcription Factor Binding Site H G00-118-929 MTH523-550 none MD400944 Matrix Mitochondrial genome transcription MTHSP1 Major H-strand promoter G00-118-908 MTH545-567 none MD400950 Mitochondrial Transcription Factor A (TCF6L2) Matrix Mitochondrial DNA Transcription TCF6L2 Mitochondrial Transcription Factor A G00-127-555 10q21 hum_10 Fahimi66; Guttler67; Alessio67 MD400951 Single-stranded DNA-binding Protein Matrix Mitochondrial DNA Replication SSBP Single-stranded DNA-binding Protein G00-386-190 7q34 hum_07 Zaheer67 MD400952 Endonuclease G Matrix RNA maturation or possibly DNA repair ENDOG Endonuclease G G00-386-191 9q34.1 hum_09 Zaheer67 MD500001 1.1.1.27 L-lactate:NAD oxidoreductase(iso) 150000 several Endahl60; Sulimovici69 MD500005 1.1.1.43 6-Phospho-D-gluconate:NAD oxidoreductase (iso) rat liver “latent” Monder63 MD500010 1.1.1.49 D-Glucose-6-phosphate:NADP oxidoreductase (iso) 305900 aka D-Glucose-6-phosphate 1-dehydrogenase aka G6PD. rat liver Avigan61 MD500015 1.1.1.51 3(or 17)-beta -Hydroxysteroid:NAD(P) oxidoreductase (NAD) "3(or 17)-beta -Hydroxysteroid dehydrogenase, guinea pig liver, ox adrenal" Brosemer65 MD500020 1.1.1.150 21-Hydroxysteroid-NAD oxidoreductase sheep liver Ohnishi66; Vitols61 MD500025 1.1.1.- 24-Dehydrocholestreol reductase rat liver Williams52a; Kensler51; Rendina59 MD500030 1.1.1.12 ’-Pyrroline-5-carboxylate:NAD oxidoreductase Locusta muscle Williams52b MD500035 1.1.2.3/4 L-Lactate:cytochrome c oxidoreductase yeast Munn74 MD500040 1.1.99.1 Choline:(acceptor) oxidoreductase aka choline dehydrogenase. rat liver Munn74 MD500045 1.2.1.8 Betaine aldehyde:NAD oxidoreductase aka Betaine-aldehyde dehydrogenase. rat liver Green54 MD500050 1.2.3.1 Aldehyde:O2 oxidoreductase pig liver Gupta85 MD500055 1.2.4.1 Pyruvate:lipoate oxidoreductase (acceptor acetylating) pig heart Waldschmidt67; Stoffel65; Green54; Crane56; Hauge56 MD500060 1.3.2.1 Butyryl-CoA:cytochrome c oxidoreductase "ox, pig liver" Sano61 MD500065 mitochondrial protein P1 de Duve55 MD500075 1.3.2.2 Acyl-CoA:cytochrome c oxidoreductase "rat, pig liver" Hawkins52; de Duve60; Aile59 MD500080 1.3.3.3 Coproporphyrinogen oxidase (decarboxylating) not "ox, guinea, pig liver" Srivastava68; Valette54 MD500085 1.3.99.- Succinate cytochrome c reductase not rat liver Noronha60 MD500092 1.4.3.6 Diamine:O2 oxidoreductase (deaminating) "rabbit liver, pig kidney" Hoskins61 MD500095 1.5.1.3 Dihydrofolate reductase "aka 5,6,7,8-Tetrahydrofolate:NAD(P) oxidoreductase aka tetrahydrofolate dehydrogenase. rat liver" de Duve55; Spiro61a; Spiro61b; Hogeboom49; Giuditta59; Kuff56; Eichel57; Hauge56 MD500096 1.5.1.4 "7,8-Dihydrofolate:NADP oxidoreductase" rat liver de Duve55; Hogeboom49; Hogeboom50; Kikuchi59 MD500100 1.5.3.1 Sarcosine:O2 oxidoreductase (demethylating) "aka sarcosine oxidase. rat liver, ""soluble""" Schnaitman68 MD500105 1.6.2.1 NADH2:cytochrome c oxidoreductase numerous Slater59; Harper62 MD500107 1.6.2.3 NADPH2:cytochrome c oxidoreductase "numerous, Ascaris muscle" Bernheim69 MD500110 1.6.4.3 NADH2:lipoamide oxidoreductase rat liver Wattiaux-De Coninck71; Cohen72 MD500115 1.6.99.5 NADH2:(acceptor) oxidoreductase "rat liver, BRAIN" Sauer60 MD500120 1.7.99.1 Ammonia:(acceptor) oxidoreductase aka HYDROXYLAMINE REDUCTASE; liver De Groot60 MD500125 1.8.3.1 Sulphite:O2 oxidoreductase 272300 "aka sulfite oxidase. rat, ox liver" Noronha60; Nakano68; Fujioka69 MD500130 1.11.1.7 Donor:H2O2 oxidoreductase 254600 peroxidase aka myeloperoxidase. ascites cells Reichard54 MD500131 1.11.1.8 Iodine:H2O2 oxidoreductase 274500 "iodide peroxidase aka iodotyrosine deiodase aka iodinase aka thyroid peroxidase, sheep thyroid" Zebe60 MD500135 2.1.2.1 L-Serine:tetrahydrofolate 10-hydroxymethyl transferase (iso) "rat, rabbit liver" Munn74 MD500140 2.1.3.2 Carbomyl phosphate:L-aspartate carbomyl transferase "aka ASPARTATE CARBAMOYLTRANSFERASE aka ASPARTATE TRANSCARBAMYLASE aka CARBAMYLASPARTOTRANSKINASE aka ATCASE. rat, rabbit liver" Schachter54; Brandt68 MD500145 2.3.1.9 Acetyl-CoA:acetyl-CoA acetyltransferase 203750 aka ACETYL-COA C-ACETYLTRANSFERASE aka ACETOACETYL-COA THIOLASE. Disease: ALPHA-METHYLACETOACETICACIDURIA. Locusta flight muscle Zebe60 MD500148 2.3.1.12 Acetk-CoA:dihydrolipoate S-acetyltransferase aka DIHYDROLIPOAMIDE S-ACETYLTRANSFERASE aka LIPOATE ACETYLTRANSFERASE aka THIOLTRANSACETYLASE A. Barnes71 MD500149 2.3.1.13 Acyl-CoA:glycine N-acyltransferase "aka GLYCINE N-ACYLTRANSFERASE. ox, rat liver" Swick65; Delbruck59 MD500155 2.3.1-??? Succinyl-CoA:glycine succinyltransferase "rat liver and kidney, NOT heart NOR brain" Swick67; Matsuzawa68; Katunuma65 MD500163 2.6.1.5 L-Tyrosine:2-oxoglutarate aminotransferase 276600 aka TYROSINE AMINOTRANSFERASE aka TYROSINE TRANSAMINASE. Disease: TYROSINEMIA II (RICHNER-HANHART SYNDROME). rat liver Rowsell72 MD500171 2.6.1.13 L-Ornithine:2-oxoacid aminotransferase 258870 aka ORNITHINE--OXO-ACID AMINOTRANSFERASE ORNITHINE AMINOTRANSFERASE. ORNITHINE KETOACID AMINOTRANSFERASE. Disease: ORNITHINEMIA WITH GYRATE ATROPHY OF CHOROID AND RETINA. liver Kosow68; Hers51; Saltman53; Crane53; Srivastava68; Mayer71 MD500177 2.6.1.19 4-Aminobutyrate aminotransferase (iso) 137150 aka 4-AMINOBUTYRATE AMINOTRANSFERASE aka GAMMA-AMINO-N- BUTYRATE TRANSAMINASE aka GABA TRANSAMINASE aka BETA- ALANINE--OXOGLUTARATE AMINOTRANSFERASE Disease: GABA- TRANSAMINASE DEFICIENCY. rat BRAIN McCormick62 MD500180 2.6.1.44 L-Alanine:glyoxylate aminotransferase (iso) "mammalian liver, kidney" Tietz58; Warner58; Bucher55 MD500185 2.7.1.1 ATP:D-hexose 6-phosphotransferase (types I & II) 235700 aka HEXOKINASE aka GLUCOKINASE aka HEXOKINASE TYPE IV. Disease: HEMOLYTIC ANEMIA DUE TO HEXOKINASE DEFICIENCY. numerous Leuthardt49; Reichard54 MD500210 2.7.1.26 ATP:riboflavin 5'-phosphotransferase aka RIBOFLAVIN KINASE aka FLAVOKINASE. rat liver Virden72 MD500224 2.7.1.40 ATP:pyruvate phosphotransferase 266200 aka PYRUVATE KINASE aka PHOSPHOENOLPYRUVATE KINASE aka PHOSPHOENOL TRANSPHOSPHORYLASE. Disease:HEMOLYTIC ANEMIA DUE TO PYRUVATE KINASE DEFICIENCY. rabbit muscle Norman65; Colomb69; Pedersen68 MD500230 2.7.2.2 ATP:carbamate phosphotransferase aka CARBAMATE KINASE. rat liver Neubert68; South68 MD500233 2.7.2.5 Carbamoyl phosphate synthase Inner membrane rat liver Clarke76; Wintersberger65; Wintersberger70; Meyer69 MD500240 2.7.4.6 ATP:nucleoside diphosphate phosphotransferase "artichoke, ox heart, liver" McCaman60; Smith70; Chojnacki63 MD500245 2.7.7.6 Nucleosidetriphosphate:RNA nucleotidyltransferase "rat liver, S. cerevisiae" Rabinowitz65; de Robertis67 MD500246 2.7.7.7 Deoxynucleosidetriphosphate:DNA deoxynucleotidyltransferase "yeast, rat liver" de Duve55; Moyle53; Ludewig51 MD500250 2.7.8.2 "CDP choline:1,2-diglyceride choline phosphotransferase" "Tetrahymena, rat BRAIN, chicken liver" Munn74 MD500255 4.6.1.1 Adenyl cyclase "rat liver, BRAIN" Norum64 MD500270 3.1.2.2 Palmitoyl-CoA hydrolase calf liver Rademaker57 MD500275 3.1.4.5 Deoxyribonucleate oligonucleotido-hydrolase rat liver Rademaker57 MD500280 3.2.1.28 Trehalose 1-glucohydrolase Sarcophaga f.m. MEM. Shepherd51; O'Donovan66 MD500285 3.4.2.1 Carboxypeptidase A rat liver Mehlman67 MD500290 3.4.16.1 Cathepsin A rat liver Haber70 MD500314 4.1.1.15 L-Glutamate 1-carboxy-lyase (iso) "mouse, human BRAIN" Bucher60 MD500330 4.1.1.31 Orthophosphate:oxaloacetate carboxy-lyase (phosphorylating) "Bryophyllum, cauliflower and pea seedlings" Bucher60 MD500331 4.1.1.32 GTP:oxaloacetate carboxy-lyase (transphosphorylating)(iso) numerous Nordie63; Bandurski56 MD500339 4.1.3.8 ATP-citrate lyase rat liver Stoffel64; Stoffel65 MD500350 5.1.2.- 3-Hydroxyacyl-CoA 3-epimerase rat liver Munn74 MD500355 5.3.3.1 3-ketosteroid delta4-delta5-isomerase human placenta none MD500360 5.3.3.8 delta3-cis-delta2-trans-enoyl-CoA isomerase rat liver Palmer66 MD500370 6.1.1- Amino acid:sRNA ligases (AMP) Nielsen49 MD500380 6.2.1- Octanoate CoA ligase ox liver Tyler92.page 178 MD500385 6.2.1- Benzoyl-CoA synthetase "guinea pig, rat liver" Ryzlak88; Tyler92.page 180 MD500390 2.3.1.37 delta-Aminolaevulinate synthetase "aka 5-aminolevulinate synthase, rat liver" Ryzlak88; Tyler92.page 180 MD500410 1.5.99.1 Sarcosine dehydrogenase rat liver Tyler92.page 199 MD500420 2.7.6.2 Thiamine pyrophosphokinase not sure it is mitochondrial Tyler92.page 210 MD500430 2.3.1.137 Octanoyltransferase aka octanoyl-CoA:L-canitine O-octanoyltransferase Tyler92.page 486 MD500435 Electron-transferring flavoprotein (ETF) Tyler92.page 241 MD500450 Dicarboxylate carrier "plant mitochondria, to transport in malate, from reduction of oxaloacetate, formed from PEP by PEP carboxylase" Tyler92.page 270 MD500455 "4.1.1.31, 32, 38, 49" PEP carboxylase "in plant cytosol, forming oxaloacetate from PEP, Mg+2" Tyler92.page 290 MD500459 ETF dehydrogenase ferriflavoprotein Tyler92.page 290 MD500460 Q-binding apoproteins evidence for existence largely circumstantial Tyler92.page 290 MD500461 QPn "located in complex I, unknown MW, ?site of Q reduction and rotenone and piericidin binding" Ruzicka74; Tyler92.page 291 MD500462 QPs "complex II, 15 kDal, reconstitutes with succinate to form s-Q reductase" Ruzicka74; Tyler92.page 290 MD500463 QPc "complex III, 15 kDal, reconstitutes with a deficient prep (bc1-IV complex of King) to form QH2-cyto c reductase. Two QPc molecules per cyto c1 are thought to be present. they might function as the Qin and Qout centers of the Q-cycle in complex III" Rieske76; Trumpower81 MD500470 1.3.99.2-3 "ETF, electron-transferring flavoprotein" reoxidized by separate ETF-ubiquinone oxidoreductase Tyler92.page 279 MD500475 1.5.5.1 ETF-ubiquinone oxidoreductase Tyler92. page 279 MD500480 Iron-sulfur protein (FeSIII-Rieske) Tyler92.page 281 MD500485 Iron-sulfur protein FeSs-3 center (4Fe4S structure) Tyler92.page 298 MD500490 ETF dehydrogenase FeSetf center (4Fe4S structure) Tyler92.page 298 MD500495 a-type cytochromes heme group contains formyl and hydrocarbon side chains not in other cytochromes Tyler92.page 301 MD500500 1.1.2.3 L-lactate:cytochrome c reductase aka cytochrome b2 Way88; Vennesland82; Tyler92.page 302 MD500505 1.1.2.5 D-lactate:cytochrome c reductase in yeast mitos none MD500510 Alternative oxidase "cyanide-insensitive, thermolabile oxidase activity of many plant mitos, abolished by quinone extraction and restored by adding ubiquinones" Papa71 MD500525 Proton-linked Pi carrier Azrolan89; Pedersen78 MD500530 Proton-linked Pyruvate Carrier none MD500535 Proton-linked Glutamate Carrier none