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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) | |||
GCDH | glutaryl-CoA dehydrogenase; 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. Isoform Short is inactive (438 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) | |||
MRPL15 | mitochondrial ribosomal protein L15 (296 aa) | |||
ATP5B | ATP synthase, H+ transporting, mitochondrial F1 complex, beta polypeptide; Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. F-type ATPases consist of two structural domains, F(1) - containing the extramembraneous catalytic core, and F(0) - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F(1) is couple [...] (529 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) | |||
USP13 | ubiquitin specific peptidase 13 (isopeptidase T-3); Deubiquitinase that mediates deubiquitination of target proteins such as BECN1, MITF, SKP2 and USP10 and is involved in various processes such as autophagy and endoplasmic reticulum- associated degradation (ERAD). Component of a regulatory loop that controls autophagy and p53/TP53 levels- mediates deubiquitination of BECN1, a key regulator of autophagy, leading to stabilize the PIK3C3/VPS34-containing complexes. Also deubiquitinates USP10, an essential regulator of p53/TP53 stability. In turn, PIK3C3/VPS34- containing complexes regula [...] (863 aa) | |||
ACAT1 | acetyl-CoA acetyltransferase 1; Plays a major role in ketone body metabolism (427 aa) | |||
MUT | methylmalonyl CoA mutase; Involved in the degradation of several amino acids, odd- chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle. MCM has different functions in other species (750 aa) | |||
MRPL49 | mitochondrial ribosomal protein L49 (166 aa) | |||
ACAA2 | acetyl-CoA acyltransferase 2; Abolishes BNIP3-mediated apoptosis and mitochondrial damage (397 aa) | |||
MRPL39 | mitochondrial ribosomal protein L39 (353 aa) | |||
SIAH2 | siah E3 ubiquitin protein ligase 2; E3 ubiquitin-protein ligase that mediates ubiquitination and subsequent proteasomal degradation of target proteins. E3 ubiquitin ligases accept ubiquitin from an E2 ubiquitin- conjugating enzyme in the form of a thioester and then directly transfers the ubiquitin to targeted substrates. Mediates E3 ubiquitin ligase activity either through direct binding to substrates or by functioning as the essential RING domain subunit of larger E3 complexes. Triggers the ubiquitin-mediated degradation of many substrates, including proteins involved in transcriptio [...] (324 aa) | |||
HMGCS1 | 3-hydroxy-3-methylglutaryl-CoA synthase 1 (soluble); This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase (520 aa) | |||
HADHB | hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit (474 aa) | |||
SLC25A21 | solute carrier family 25 (mitochondrial oxoadipate carrier), member 21; Transports C5-C7 oxodicarboxylates across the inner membranes of mitochondria. Can transport 2-oxoadipate, 2- oxoglutarate, adipate, glutarate, and to a lesser extent, pimelate, 2-oxopimelate, 2-aminoadipate, oxaloacetate, and citrate (299 aa) | |||
MRPL40 | mitochondrial ribosomal protein L40 (206 aa) | |||
ACAA1 | acetyl-CoA acyltransferase 1 (424 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) | |||
UBC | ubiquitin C (685 aa) | |||
ACAT2 | acetyl-CoA acetyltransferase 2 (397 aa) | |||
HMGCS2 | 3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial); This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase (508 aa) | |||
OGDHL | oxoglutarate dehydrogenase-like (1010 aa) | |||
HMGCL | 3-hydroxymethyl-3-methylglutaryl-CoA lyase; Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism (325 aa) | |||
UIMC1 | ubiquitin interaction motif containing 1 (719 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) |