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ECHS1 | Enoyl-CoA hydratase, short chain 1; Belongs to the enoyl-CoA hydratase/isomerase family. (291 aa) | ||||
ACACA | Acetyl-CoA carboxylase 1; Cytosolic enzyme that catalyzes the carboxylation of acetyl- CoA to malonyl-CoA, the first and rate-limiting step of de novo fatty acid biosynthesis. This is a 2 steps reaction starting with the ATP- dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain followed by the transfer of the carboxyl group from carboxylated biotin to acetyl-CoA. (2257 aa) | ||||
ALDH6A1 | Aldehyde dehydrogenase 6 family member A1. (536 aa) | ||||
LDHAL6B | L-lactate dehydrogenase; Belongs to the LDH/MDH superfamily. (381 aa) | ||||
PCCA | Propionyl-CoA carboxylase subunit alpha. (646 aa) | ||||
ACAT2 | Acetyl-CoA acetyltransferase 2; Belongs to the thiolase-like superfamily. Thiolase family. (432 aa) | ||||
ABAT | 4-aminobutyrate aminotransferase; Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family. (500 aa) | ||||
ACSS1 | Acetyl-coenzyme A synthetase. (579 aa) | ||||
DLD | Dihydrolipoyl dehydrogenase. (509 aa) | ||||
BCKDHA | 2-oxoisovalerate dehydrogenase subunit alpha; The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). (468 aa) | ||||
ECHDC1 | Ethylmalonyl-CoA decarboxylase 1; Belongs to the enoyl-CoA hydratase/isomerase family. (307 aa) | ||||
PCCB | Propionyl-CoA carboxylase subunit beta. (550 aa) | ||||
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. (518 aa) | ||||
LDHA | L-lactate dehydrogenase; Belongs to the LDH/MDH superfamily. (361 aa) | ||||
MLYCD | Malonyl-CoA decarboxylase. (353 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. (432 aa) | ||||
ACSS2 | Acyl-CoA synthetase short chain family member 2. (714 aa) | ||||
ACAT1 | Acetyl-CoA acetyltransferase 1; Belongs to the thiolase-like superfamily. Thiolase family. (427 aa) | ||||
MMUT | Methylmalonyl-CoA mutase. (755 aa) | ||||
ACSS3 | Acyl-CoA synthetase short chain family member 3. (678 aa) | ||||
MCEE | Methylmalonyl-CoA epimerase. (179 aa) | ||||
HIBCH | 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite; Belongs to the enoyl-CoA hydratase/isomerase family. (386 aa) | ||||
ACACB | Acetyl-CoA carboxylase beta. (2454 aa) | ||||
LOC114118359 | L-lactate dehydrogenase; Belongs to the LDH/MDH superfamily. (339 aa) | ||||
HADHA | Enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase; Belongs to the enoyl-CoA hydratase/isomerase family. (763 aa) | ||||
LDHB | L-lactate dehydrogenase; Belongs to the LDH/MDH superfamily. (337 aa) | ||||
SUCLG1 | Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial; Succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of either ATP or GTP and thus represents the only step of substrate-level phosphorylation in the TCA. The alpha subunit of the enzyme binds the substrates coenzyme A and phosphate, while succinate binding and specificity for either ATP or GTP is provided by different beta subunits. (350 aa) | ||||
EHHADH | Enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase. (723 aa) |