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DLD | dihydrolipoamide dehydrogenase; Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction (509 aa) | |||
ACO2 | aconitase 2, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis-aconitate (By similarity) (780 aa) | |||
BCKDK | branched chain ketoacid dehydrogenase kinase; Catalyzes the phosphorylation and inactivation of the branched-chain alpha-ketoacid dehydrogenase complex, the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Key enzyme that regulate the activity state of the BCKD complex (By similarity) (412 aa) | |||
OGDH | oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide); The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components- 2- oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3) (1023 aa) | |||
PDHX | pyruvate dehydrogenase complex, component X; Required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex (501 aa) | |||
MRPS2 | mitochondrial ribosomal protein S2 (296 aa) | |||
ACLY | ATP citrate lyase; ATP citrate-lyase is the primary enzyme responsible for the synthesis of cytosolic acetyl-CoA in many tissues. Has a central role in de novo lipid synthesis. In nervous tissue it may be involved in the biosynthesis of acetylcholine (1101 aa) | |||
MRPS36 | mitochondrial ribosomal protein S36 (103 aa) | |||
MRPL15 | mitochondrial ribosomal protein L15 (296 aa) | |||
DHTKD1 | dehydrogenase E1 and transketolase domain containing 1; The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components- 2- oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3) (By similarity) (919 aa) | |||
BCKDHA | branched chain keto acid dehydrogenase E1, alpha polypeptide; 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) (445 aa) | |||
DLAT | dihydrolipoamide S-acetyltransferase; 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 (647 aa) | |||
MRPL13 | mitochondrial ribosomal protein L13 (178 aa) | |||
PDHB | pyruvate dehydrogenase (lipoamide) beta; 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 (359 aa) | |||
CYC1 | cytochrome c-1; This is the heme-containing component of the cytochrome b-c1 complex, which accepts electrons from Rieske protein and transfers electrons to cytochrome c in the mitochondrial respiratory chain (325 aa) | |||
BCKDHB | branched chain keto acid dehydrogenase E1, beta polypeptide; 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) (392 aa) | |||
GCSH | glycine cleavage system protein H (aminomethyl carrier); The glycine cleavage system catalyzes the degradation of glycine. The H protein shuttles the methylamine group of glycine from the P protein to the T protein (173 aa) | |||
DLST | dihydrolipoamide S-succinyltransferase (E2 component of 2-oxo-glutarate complex); The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of 3 enzymatic components- 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3) (453 aa) | |||
LIPT1 | lipoyltransferase 1; Catalyzes the transfer of the lipoyl group from lipoyl- AMP to the specific lysine residue of lipoyl domains of lipoate- dependent enzymes (By similarity) (373 aa) | |||
NDUFS2 | NADH dehydrogenase (ubiquinone) Fe-S protein 2, 49kDa (NADH-coenzyme Q reductase); Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity) (463 aa) | |||
DBT | dihydrolipoamide branched chain transacylase E2; 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) (482 aa) | |||
OGDHL | oxoglutarate dehydrogenase-like (1010 aa) | |||
PDHA1 | pyruvate dehydrogenase (lipoamide) alpha 1 (428 aa) | |||
SUCLG1 | succinate-CoA ligase, alpha subunit; Catalyzes the ATP- or GTP-dependent ligation of succinate and CoA to form succinyl-CoA. The nature of the beta subunit determines the nucleotide specificity (By similarity) (346 aa) | |||
SCFD1 | sec1 family domain containing 1; Plays a role in SNARE-pin assembly and Golgi-to-ER retrograde transport via its interaction with COG4. Involved in vesicular transport between the endoplasmic reticulum and the Golgi (By similarity) (642 aa) | |||
SUCLG2 | succinate-CoA ligase, GDP-forming, beta subunit; Catalyzes the GTP-dependent ligation of succinate and CoA to form succinyl-CoA (By similarity) (440 aa) |