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| | SUCLA2 | Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial; ATP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of ATP and thus represents the only step of substrate-level phosphorylation in the TCA. The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit. (463 aa) |
| | ACOT6 | Uncharacterized protein. (421 aa) |
| | ACAT1 | Acetyl-CoA acetyltransferase 1; Belongs to the thiolase-like superfamily. Thiolase family. (426 aa) |
| | ACSS1 | Acetyl-coenzyme A synthetase. (703 aa) |
| | ACACA | Acetyl-CoA carboxylase alpha. (2383 aa) |
| | PIPOX | Pipecolic acid and sarcosine oxidase. (401 aa) |
| | HTD2 | MaoC-like domain-containing protein. (180 aa) |
| | ACOT12 | Acyl-coenzyme A thioesterase 12 isoform X1. (559 aa) |
| | FAR2 | Fatty acyl-CoA reductase; Catalyzes the reduction of fatty acyl-CoA to fatty alcohols. (515 aa) |
| | ELOVL1 | Elongation of very long chain fatty acids protein 1; Catalyzes the first and rate-limiting reaction of the four reactions that constitute the long-chain fatty acids elongation cycle. This endoplasmic reticulum-bound enzymatic process allows the addition of 2 carbons to the chain of long- and very long-chain fatty acids (VLCFAs) per cycle. Condensing enzyme that exhibits activity toward saturated C18 to C26 acyl-CoA substrates, with the highest activity towards C22:0 acyl-CoA. May participate to the production of both saturated and monounsaturated VLCFAs of different chain lengths that [...] (314 aa) |
| | PANK2 | Pantothenate kinase 2. (451 aa) |
| | GPAT4 | PlsC domain-containing protein. (457 aa) |
| | THEM5 | Acyl-coenzyme A thioesterase THEM5. (247 aa) |
| | PMVK | Phosphomevalonate kinase. (192 aa) |
| | PPCS | Phosphopantothenoylcysteine synthetase. (311 aa) |
| | ELOVL4 | Elongation of very long chain fatty acids protein 4; Catalyzes the first and rate-limiting reaction of the four reactions that constitute the long-chain fatty acids elongation cycle. This endoplasmic reticulum-bound enzymatic process allows the addition of 2 carbons to the chain of long- and very long-chain fatty acids (VLCFAs) per cycle. Condensing enzyme that specifically elongates C24:0 and C26:0 acyl-CoAs. May participate to the production of saturated and monounsaturated VLCFAs of different chain lengths that are involved in multiple biological processes as precursors of membrane [...] (321 aa) |
| | LOC110255172 | Uncharacterized protein. (418 aa) |
| | DLST | 2-oxoglutarate dehydrogenase E2 component (dihydrolipoamide succinyltransferase); Dihydrolipoamide succinyltransferase (E2) component of the 2- oxoglutarate dehydrogenase complex. The 2- oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). The 2- oxoglutarate dehydrogenase complex is mainly active in the mitochondrion (By similarity). A fraction of the 2-oxoglutarate dehydrogenase complex also localizes in the nucleus and is required for lysine succinylation of histones: associates with KAT2A on chromatin and provides succinyl [...] (446 aa) |
| | LOC100515185 | Uncharacterized protein. (423 aa) |
| | PDHB | Pyruvate dehydrogenase E1 component subunit beta; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2. (361 aa) |
| | NUDT7 | Nudix hydrolase 7. (237 aa) |
| | ACOT9 | Acyl-CoA thioesterase 9. (449 aa) |
| | PDHX | Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex. (498 aa) |
| | DCAKD | Dephospho-CoA kinase domain containing. (264 aa) |
| | ACOT4 | Acyl-CoA thioesterase 4. (507 aa) |
| | FITM2 | Fat storage-inducing transmembrane protein 2; Plays an important role in lipid droplet accumulation. Plays a role in the regulation of cell morphology and cytoskeletal organization; Belongs to the FIT family. (283 aa) |
| | ACACB | Biotin carboxylation domain-containing protein. (229 aa) |
| | NUDT19 | Nudix hydrolase 19. (322 aa) |
| | DLAT | Acetyltransferase component of pyruvate dehydrogenase complex; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). (667 aa) |
| | TDO2 | Tryptophan 2,3-dioxygenase; Heme-dependent dioxygenase that catalyzes the oxidative cleavage of the L-tryptophan (L-Trp) pyrrole ring and converts L- tryptophan to N-formyl-L-kynurenine. Catalyzes the oxidative cleavage of the indole moiety. (486 aa) |
| | MPC2 | Mitochondrial pyruvate carrier; Mediates the uptake of pyruvate into mitochondria. Belongs to the mitochondrial pyruvate carrier (MPC) (TC 2.A.105) family. (127 aa) |
| | DGAT1 | Diacylglycerol O-acyltransferase 1; Belongs to the membrane-bound acyltransferase family. (665 aa) |
| | CROT | Carn_acyltransf domain-containing protein; Belongs to the carnitine/choline acetyltransferase family. (612 aa) |
| | ACOT11 | Acyl-CoA thioesterase 11. (598 aa) |
| | ACSL3 | Acyl-CoA synthetase long chain family member 3. (729 aa) |
| | AASS | Aminoadipate-semialdehyde synthase. (961 aa) |
| | HMGCR | 3-hydroxy-3-methylglutaryl-coenzyme A reductase; Transmembrane glycoprotein that is the rate-limiting enzyme in cholesterol biosynthesis as well as in the biosynthesis of nonsterol isoprenoids that are essential for normal cell function including ubiquinone and geranylgeranyl proteins. (887 aa) |
| | PPCDC | Phosphopantothenoylcysteine decarboxylase. (220 aa) |
| | FAR1 | Fatty acyl-CoA reductase; Catalyzes the reduction of fatty acyl-CoA to fatty alcohols. (535 aa) |
| | ACSS2 | Acetyl-coenzyme A synthetase, cytoplasmic isoform X1. (714 aa) |
| | NUDT8 | Nudix hydrolase domain-containing protein. (140 aa) |
| | ACSL4 | Acyl-CoA synthetase long chain family member 4. (711 aa) |
| | GCDH | Glutaryl-CoA dehydrogenase, mitochondrial; Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L- hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. (438 aa) |
| | HSD17B4 | Peroxisomal multifunctional enzyme type 2 isoform 2. (737 aa) |
| | ACSL5 | Acyl-CoA synthetase long chain family member 5. (705 aa) |
| | ACOT8 | Acyl-coenzyme A thioesterase 8. (317 aa) |
| | ACSM5 | Acyl-coenzyme A synthetase ACSM5, mitochondrial isoform 1. (579 aa) |
| | ACLY | ATP-citrate synthase; Catalyzes the cleavage of citrate into oxaloacetate and acetyl-CoA, the latter serving as common substrate for de novo cholesterol and fatty acid synthesis; In the N-terminal section; belongs to the succinate/malate CoA ligase beta subunit family. (1130 aa) |
| | HMGCS1 | 3-hydroxy-3-methylglutaryl coenzyme A synthase; This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase. (576 aa) |
| | OGDH | Oxoglutarate dehydrogenase. (1035 aa) |
| | ABCD1 | ATP binding cassette subfamily D member 1. (732 aa) |
| | OXSM | 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial; May play a role in the biosynthesis of lipoic acid as well as longer chain fatty acids required for optimal mitochondrial function. Belongs to the thiolase-like superfamily. Beta-ketoacyl-ACP synthases family. (460 aa) |
| | GPAM | Glycerol-3-phosphate acyltransferase 1, mitochondrial; Esterifies acyl-group from acyl-ACP to the sn-1 position of glycerol-3-phosphate, an essential step in glycerolipid biosynthesis. (825 aa) |
| | ELOVL3 | Elongation of very long chain fatty acids protein 3; Catalyzes the first and rate-limiting reaction of the four reactions that constitute the long-chain fatty acids elongation cycle. This endoplasmic reticulum-bound enzymatic process allows the addition of 2 carbons to the chain of long- and very long-chain fatty acids (VLCFAs) per cycle. Condensing enzyme with higher activity toward C18 acyl-CoAs, especially C18:0 acyl-CoAs. May participate to the production of saturated and monounsaturated VLCFAs of different chain lengths that are involved in multiple biological processes as precurs [...] (270 aa) |
| | MVK | Mevalonate kinase. (454 aa) |
| | ACSM2B | Uncharacterized protein. (578 aa) |
| | ACSM3 | Acyl-CoA synthetase medium chain family member 3. (581 aa) |
| | PANK1 | Pantothenate kinase 1. (648 aa) |
| | BAAT | Bile acid-CoA:amino acid N-acyltransferase. (407 aa) |
| | PANK3 | Pantothenate kinase 3. (370 aa) |
| | ACSL1 | Acyl-CoA synthetase long chain family member 1. (771 aa) |
| | SULT1B1 | Sulfotransferase; Belongs to the sulfotransferase 1 family. (296 aa) |
| | PAPSS1 | 3'-phosphoadenosine 5'-phosphosulfate synthase 1. (808 aa) |
| | ENPP1 | Ectonucleotide pyrophosphatase/phosphodiesterase 1. (878 aa) |
| | MVD | Mevalonate diphosphate decarboxylase. (474 aa) |
| | ELOVL6 | Elongation of very long chain fatty acids protein 6; Catalyzes the first and rate-limiting reaction of the four reactions that constitute the long-chain fatty acids elongation cycle. This endoplasmic reticulum-bound enzymatic process allows the addition of 2 carbons to the chain of long- and very long-chain fatty acids (VLCFAs) per cycle. Condensing enzyme that elongates fatty acids with 12, 14 and 16 carbons with higher activity toward C16:0 acyl-CoAs. Catalyzes the synthesis of unsaturated C16 long chain fatty acids and, to a lesser extent, C18:0 and those with low desaturation degre [...] (265 aa) |
| | SUCLG2 | Succinate--CoA ligase [GDP-forming] subunit beta, mitochondrial; GTP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of GTP and thus represents the only step of substrate-level phosphorylation in the TCA. The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit. (457 aa) |
| | SULT1E1 | Sulfotransferase; Belongs to the sulfotransferase 1 family. (296 aa) |
| | MCEE | Methylmalonyl-CoA epimerase. (173 aa) |
| | CCDC30 | Uncharacterized protein. (997 aa) |
| | HMGCS2 | Hydroxymethylglutaryl-CoA synthase, mitochondrial; This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase; Belongs to the thiolase-like superfamily. HMG-CoA synthase family. (508 aa) |
| | PAPSS2 | Bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthase 2 isoform b. (620 aa) |
| | PDHA1 | Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle. (396 aa) |
| | ACSM4 | Acyl-CoA synthetase medium chain family member 4. (580 aa) |
| | ELOVL5 | Elongation of very long chain fatty acids protein 5; Catalyzes the first and rate-limiting reaction of the four reactions that constitute the long-chain fatty acids elongation cycle. This endoplasmic reticulum-bound enzymatic process allows the addition of 2 carbons to the chain of long- and very long-chain fatty acids (VLCFAs) per cycle. Condensing enzyme that acts specifically toward polyunsaturated acyl-CoA with the higher activity toward C18:3(n-6) acyl-CoA. May participate to the production of monounsaturated and of polyunsaturated VLCFAs of different chain lengths that are involv [...] (325 aa) |
| | PANK4 | 4'-phosphopantetheine phosphatase; May play a role in the physiological regulation of coenzyme A (CoA) intracellular levels. The phosphatase activity shows preference for normal or oxidatively damaged intermediates of 4'- phosphopantetheine, which provides strong indirect evidence that the phosphatase activity pre-empts damage in the CoA pathway. (773 aa) |
| | ACSL6 | Long-chain-fatty-acid--CoA ligase 6 isoform X1. (722 aa) |
| | MLYCD | Malonyl-CoA decarboxylase. (499 aa) |
| | COASY | Phosphopantetheine adenylyltransferase; Bifunctional enzyme that catalyzes the fourth and fifth sequential steps of CoA biosynthetic pathway. The fourth reaction is catalyzed by the phosphopantetheine adenylyltransferase, coded by the coaD domain; the fifth reaction is catalyzed by the dephospho-CoA kinase, coded by the coaE domain. May act as a point of CoA biosynthesis regulation. (617 aa) |
| | ACOT7 | Acyl-CoA thioesterase 7. (406 aa) |
| | HDDC3 | HD domain containing 3. (233 aa) |
