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PCK2 | Phosphoenolpyruvate carboxykinase [GTP], mitochondrial; Catalyzes the conversion of oxaloacetate (OAA) to phosphoenolpyruvate (PEP), the rate-limiting step in the metabolic pathway that produces glucose from lactate and other precursors derived from the citric acid cycle; Belongs to the phosphoenolpyruvate carboxykinase [GTP] family (640 aa) | |||
IDH3G | Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial; Regulatory subunit which plays a role in the allosteric regulation of the enzyme catalyzing the decarboxylation of isocitrate (ICT) into alpha-ketoglutarate. The heterodimer composed of the alpha (IDH3A) and beta (IDH3B) subunits and the heterodimer composed of the alpha (IDH3A) and gamma (IDH3G) subunits, have considerable basal activity but the full activity of the heterotetramer (containing two subunits of IDH3A, one of IDH3B and one of IDH3G) requires the assembly and cooperative function of both heterodimers (393 aa) | |||
OGDH | 2-oxoglutarate dehydrogenase, mitochondrial; 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) | |||
ACLY | ATP-citrate synthase; ATP-citrate synthase 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; In the N-terminal section; belongs to the succinate/malate CoA ligase beta subunit family (1101 aa) | |||
FOLH1 | Glutamate carboxypeptidase 2; Has both folate hydrolase and N-acetylated-alpha-linked- acidic dipeptidase (NAALADase) activity. Has a preference for tri- alpha-glutamate peptides. In the intestine, required for the uptake of folate. In the brain, modulates excitatory neurotransmission through the hydrolysis of the neuropeptide, N- aceylaspartylglutamate (NAAG), thereby releasing glutamate. Involved in prostate tumor progression (750 aa) | |||
ASPA | Aspartoacylase; Catalyzes the deacetylation of N-acetylaspartic acid (NAA) to produce acetate and L-aspartate. NAA occurs in high concentration in brain and its hydrolysis NAA plays a significant part in the maintenance of intact white matter. In other tissues it act as a scavenger of NAA from body fluids (313 aa) | |||
PNKD | Probable hydrolase PNKD; Probable hydrolase that plays an aggravative role in the development of cardiac hypertrophy via activation of the NF-kappa- B signaling pathway; Belongs to the metallo-beta-lactamase superfamily. Glyoxalase II family (385 aa) | |||
LACTB2 | Endoribonuclease LACTB2; Endoribonuclease; cleaves preferentially 3’ to purine- pyrimidine dinucleotide motifs in single-stranded RNA. The cleavage product contains a free 3’ -OH group. Has no activity with double-stranded RNA or DNA. Required for normal mitochondrial function and cell viability; Belongs to the metallo-beta-lactamase superfamily. Glyoxalase II family (288 aa) | |||
ETHE1 | Persulfide dioxygenase ETHE1, mitochondrial; Sulfur dioxygenase that plays an essential role in hydrogen sulfide catabolism in the mitochondrial matrix. Hydrogen sulfide (H(2)S) is first oxidized by SQRDL, giving rise to cysteine persulfide residues. ETHE1 consumes molecular oxygen to catalyze the oxidation of the persulfide, once it has been transferred to a thiophilic acceptor, such as glutathione (R-SSH). Plays an important role in metabolic homeostasis in mitochondria by metabolizing hydrogen sulfide and preventing the accumulation of supraphysiological H(2)S levels that have toxic [...] (254 aa) | |||
IDH3A | Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial; Catalytic subunit of the enzyme which catalyzes the decarboxylation of isocitrate (ICT) into alpha-ketoglutarate. The heterodimer composed of the alpha (IDH3A) and beta (IDH3B) subunits and the heterodimer composed of the alpha (IDH3A) and gamma (IDH3G) subunits, have considerable basal activity but the full activity of the heterotetramer (containing two subunits of IDH3A, one of IDH3B and one of IDH3G) requires the assembly and cooperative function of both heterodimers (366 aa) | |||
MBLAC2 | Metallo-beta-lactamase domain containing 2; Belongs to the metallo-beta-lactamase superfamily. Glyoxalase II family (279 aa) | |||
PCK1 | Phosphoenolpyruvate carboxykinase, cytosolic [GTP]; Catalyzes the conversion of oxaloacetate (OAA) to phosphoenolpyruvate (PEP), the rate-limiting step in the metabolic pathway that produces glucose from lactate and other precursors derived from the citric acid cycle; Belongs to the phosphoenolpyruvate carboxykinase [GTP] family (622 aa) | |||
MDH2 | Malate dehydrogenase, mitochondrial; Malate dehydrogenase 2 (338 aa) | |||
IDH2 | Isocitrate dehydrogenase [NADP], mitochondrial; Plays a role in intermediary metabolism and energy production. It may tightly associate or interact with the pyruvate dehydrogenase complex; Belongs to the isocitrate and isopropylmalate dehydrogenases family (452 aa) | |||
CS | Citrate synthase, mitochondrial; Citrate synthase (466 aa) | |||
RIMKLB | Beta-citrylglutamate synthase B; Catalyzes the synthesis of beta-citryl-L-glutamate and N-acetyl-L-aspartyl-L-glutamate. Beta-citryl-L-glutamate is synthesized more efficiently than N-acetyl-L-aspartyl-L-glutamate (386 aa) | |||
ALDH18A1 | Delta-1-pyrroline-5-carboxylate synthase; Bifunctional enzyme that converts glutamate to glutamate 5-semialdehyde, an intermediate in the biosynthesis of proline, ornithine and arginine (795 aa) | |||
OGDHL | 2-oxoglutarate dehydrogenase-like, mitochondrial; Oxoglutarate dehydrogenase like; Belongs to the alpha-ketoglutarate dehydrogenase family (1010 aa) | |||
IDH3B | Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial; Plays a structural role to facilitate the assembly and ensure the full activity of the enzyme catalyzing the decarboxylation of isocitrate (ICT) into alpha-ketoglutarate. The heterodimer composed of the alpha (IDH3A) and beta (IDH3B) subunits and the heterodimer composed of the alpha (IDH3A) and gamma (IDH3G) subunits, have considerable basal activity but the full activity of the heterotetramer (containing two subunits of IDH3A, one of IDH3B and one of IDH3G) requires the assembly and cooperative function of both heterodimers (385 aa) | |||
HAGHL | Hydroxyacylglutathione hydrolase-like protein; Hydrolase acting on ester bonds (282 aa) | |||
PC | Pyruvate carboxylase, mitochondrial; Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate (1178 aa) | |||
HAGH | Hydroxyacylglutathione hydrolase, mitochondrial; Thiolesterase that catalyzes the hydrolysis of S-D- lactoyl-glutathione to form glutathione and D-lactic acid; Belongs to the metallo-beta-lactamase superfamily. Glyoxalase II family (308 aa) | |||
IDH1 | Isocitrate dehydrogenase 1, cytosolic (414 aa) | |||
NAT8L | N-acetylaspartate synthetase; Plays a role in the regulation of lipogenesis by producing N-acetylaspartate acid (NAA), a brain-specific metabolite. NAA occurs in high concentration in brain and its hydrolysis plays a significant part in the maintenance of intact white matter. Promotes dopamine uptake by regulating TNF-alpha expression. Attenuates methamphetamine-induced inhibition of dopamine uptake; Belongs to the camello family (302 aa) | |||
RIMKLA | N-acetylaspartylglutamate synthase A; Catalyzes the synthesis of N-acetyl-L-aspartyl-L- glutamate (NAAG) and N-acetyl-L-aspartyl-L-glutamyl-L-glutamate; Belongs to the RimK family (391 aa) | |||
MDH1 | Malate dehydrogenase, cytoplasmic; Malate dehydrogenase 1; Belongs to the LDH/MDH superfamily. MDH type 2 family (352 aa) |