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| DLD | Dihydrolipoyl dehydrogenase, mitochondrial; Lipoamide dehydrogenase is a component of the glycine cleavage system as well as an E3 component of three alpha-ketoacid dehydrogenase complexes (pyruvate-, alpha-ketoglutarate-, and branched-chain amino acid-dehydrogenase complex). In monomeric form has additional moonlighting function as serine protease. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction (By similarity) (509 aa) | |||
| ACO2 | Aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis-aconitate; Belongs to the aconitase/IPM isomerase family (780 aa) | |||
| GCLC | Glutamate-cysteine ligase catalytic subunit (637 aa) | |||
| AGXT2 | Alanine--glyoxylate aminotransferase 2, mitochondrial; Can metabolize asymmetric dimethylarginine (ADMA) via transamination to alpha-keto-delta-(NN-dimethylguanidino) valeric acid (DMGV). ADMA is a potent inhibitor of nitric-oxide (NO) synthase, and this activity provides mechanism through which the kidney regulates blood pressure; Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family (514 aa) | |||
| CAT | Catalase; Occurs in almost all aerobically respiring organisms and serves to protect cells from the toxic effects of hydrogen peroxide. Promotes growth of cells including T-cells, B-cells, myeloid leukemia cells, melanoma cells, mastocytoma cells and normal and transformed fibroblast cells; Belongs to the catalase family (527 aa) | |||
| SDS | L-serine dehydratase/L-threonine deaminase; Serine dehydratase (328 aa) | |||
| ILVBL | Acetolactate synthase-like protein; ilvB acetolactate synthase like (632 aa) | |||
| BCKDHA | 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; 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) | |||
| BCAM | Basal cell adhesion molecule; Laminin alpha-5 receptor. May mediate intracellular signaling; Blood group antigens (628 aa) | |||
| LDHAL6A | L-lactate dehydrogenase A-like 6A; Displays an lactate dehydrogenase activity. Significantly increases the transcriptional activity of JUN, when overexpressed (332 aa) | |||
| LDHAL6B | Lactate dehydrogenase A like 6B (381 aa) | |||
| BCAT2 | Branched-chain-amino-acid aminotransferase, mitochondrial; Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine. May also function as a transporter of branched chain alpha-keto acids; Belongs to the class-IV pyridoxal-phosphate-dependent aminotransferase family (392 aa) | |||
| HACL1 | 2-hydroxyacyl-CoA lyase 1; Catalyzes a carbon-carbon cleavage reaction; cleaves a 2-hydroxy-3-methylacyl-CoA into formyl-CoA and a 2-methyl-branched fatty aldehyde; Belongs to the TPP enzyme family (578 aa) | |||
| C11orf54 | Ester hydrolase C11orf54; Exhibits ester hydrolase activity on the substrate p- nitrophenyl acetate (315 aa) | |||
| GCLM | Glutamate-cysteine ligase modifier subunit; Belongs to the aldo/keto reductase family. Glutamate-- cysteine ligase light chain subfamily (274 aa) | |||
| UFM1 | Ubiquitin-fold modifier 1; Ubiquitin-like modifier which can be covalently attached via an isopeptide bond to substrate proteins as a monomer or a lysine-linked polymer. The so-called ufmylation, requires the UFM1-activating E1 enzyme UBA5, the UFM1-conjugating E2 enzyme UFC1, and the UFM1-ligase E3 enzyme UFL1. This post-translational modification on lysine residues of proteins may play a crucial role in a number of cellular processes. TRIP4 ufmylation may for instance play a role in nuclear receptors-mediated transcription. Other substrates may include DDRGK1 with which it may play a [...] (103 aa) | |||
| LIPT1 | Lipoyltransferase 1, mitochondrial; Catalyzes the transfer of the lipoyl group from lipoyl- AMP to the specific lysine residue of lipoyl domains of lipoate- dependent enzymes; Belongs to the LplA family (373 aa) | |||
| LDHB | Lactate dehydrogenase B; Belongs to the LDH/MDH superfamily. LDH family (334 aa) | |||
| SDSL | Serine dehydratase-like; Has low serine dehydratase and threonine dehydratase activity (329 aa) | |||
| FAM86A | Protein-lysine N-methyltransferase EEF2KMT; Catalyzes the trimethylation of eukaryotic elongation factor 2 (EEF2) on ’Lys-525’ (330 aa) | |||
| LDHC | L-lactate dehydrogenase C chain; Possible role in sperm motility (332 aa) | |||
| BCAT1 | Branched-chain-amino-acid aminotransferase, cytosolic; Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine (398 aa) | |||
| LDHA | Lactate dehydrogenase A; Belongs to the LDH/MDH superfamily. LDH family (361 aa) | |||
| ETFA | Electron transfer flavoprotein subunit alpha, mitochondrial; Heterodimeric electron transfer flavoprotein that accepts electrons from several mitochondrial dehydrogenases, including acyl-CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase. It transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase). Required for normal mitochondrial fatty acid oxidation and normal amino acid metabolism (333 aa) | |||
| IL4I1 | L-amino-acid oxidase; Lysosomal L-amino-acid oxidase with highest specific activity with phenylalanine. May play a role in lysosomal antigen processing and presentation (By similarity); Belongs to the flavin monoamine oxidase family. FIG1 subfamily (589 aa) | |||
| MRM1 | rRNA methyltransferase 1, mitochondrial; S-adenosyl-L-methionine-dependent 2’-O-ribose methyltransferase that catalyzes the formation of 2’-O- methylguanosine at position 1145 (Gm1145) in the 16S mitochondrial large subunit ribosomal RNA (mtLSU rRNA), a universally conserved modification in the peptidyl transferase domain of the mtLSU rRNA (353 aa) | |||
