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| 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) | |||
| EHHADH | Peroxisomal bifunctional enzyme; enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase; In the C-terminal section; belongs to the 3- hydroxyacyl-CoA dehydrogenase family (723 aa) | |||
| ACSS2 | Acetyl-coenzyme A synthetase, cytoplasmic; Activates acetate so that it can be used for lipid synthesis or for energy generation; Acyl-CoA synthetase family (714 aa) | |||
| FASTKD3 | FAST kinase domain-containing protein 3, mitochondrial; Required for normal mitochondrial respiration; Belongs to the FAST kinase family (662 aa) | |||
| HIBADH | 3-hydroxyisobutyrate dehydrogenase, mitochondrial; 3-hydroxyisobutyrate dehydrogenase (336 aa) | |||
| SOD1 | Superoxide dismutase [Cu-Zn]; Destroys radicals which are normally produced within the cells and which are toxic to biological systems (154 aa) | |||
| NDUFB11 | NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial; Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in 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; NADH-ubiquinone oxidoreductase supernumerary subunits (163 aa) | |||
| PDXK | Pyridoxal kinase; Required for synthesis of pyridoxal-5-phosphate from vitamin B6; Belongs to the pyridoxine kinase family (312 aa) | |||
| CNIH2 | Protein cornichon homolog 2; Regulates the trafficking and gating properties of AMPA- selective glutamate receptors (AMPARs). Promotes their targeting to the cell membrane and synapses and modulates their gating properties by regulating their rates of activation, deactivation and desensitization. Blocks CACNG8-mediated resensitization of AMPA receptors; Belongs to the cornichon family (160 aa) | |||
| ACSS1 | Acetyl-coenzyme A synthetase 2-like, mitochondrial; Important for maintaining normal body temperature during fasting and for energy homeostasis. Essential for energy expenditure under ketogenic conditions (By similarity). Converts acetate to acetyl-CoA so that it can be used for oxidation through the tricarboxylic cycle to produce ATP and CO(2); Belongs to the ATP-dependent AMP-binding enzyme family (689 aa) | |||
| HIBCH | 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA (386 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). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component (482 aa) | |||
| ACADM | Medium-chain specific acyl-CoA dehydrogenase, mitochondrial; Acyl-CoA dehydrogenase specific for acyl chain lengths of 4 to 16 that catalyzes the initial step of fatty acid beta- oxidation. Utilizes the electron transfer flavoprotein (ETF) as an electron acceptor to transfer electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase) (454 aa) | |||
| TRIM63 | E3 ubiquitin-protein ligase TRIM63; E3 ubiquitin ligase. Mediates the ubiquitination and subsequent proteasomal degradation of CKM, GMEB1 and HIBADH. Regulates the proteasomal degradation of muscle proteins under amino acid starvation, where muscle protein is catabolized to provide other organs with amino acids. Inhibits de novo skeletal muscle protein synthesis under amino acid starvation. Regulates proteasomal degradation of cardiac troponin I/TNNI3 and probably of other sarcomeric-associated proteins. May play a role in striated muscle atrophy and hypertrophy by regulating an anti- [...] (353 aa) | |||
| AOX1 | Aldehyde oxidase; Oxidase with broad substrate specificity, oxidizing aromatic azaheterocycles, such as N1-methylnicotinamide, N- methylphthalazinium and phthalazine, as well as aldehydes, such as benzaldehyde, retinal, pyridoxal, and vanillin. Plays a key role in the metabolism of xenobiotics and drugs containing aromatic azaheterocyclic substituents. Participates in the bioactivation of prodrugs such as famciclovir, catalyzing the oxidation step from 6-deoxypenciclovir to penciclovir, which is a potent antiviral agent. Is probably involved in the regulation of reactive oxygen species [...] (1338 aa) | |||
| PCCA | propionyl-CoA carboxylase alpha subunit (728 aa) | |||
| ALDH1B1 | Aldehyde dehydrogenase X, mitochondrial; ALDHs play a major role in the detoxification of alcohol-derived acetaldehyde. They are involved in the metabolism of corticosteroids, biogenic amines, neurotransmitters, and lipid peroxidation (517 aa) | |||
| AMELX | Amelogenin, X isoform; Plays a role in biomineralization. Seems to regulate the formation of crystallites during the secretory stage of tooth enamel development. Thought to play a major role in the structural organization and mineralization of developing enamel; Belongs to the amelogenin family (205 aa) | |||
| ABAT | 4-aminobutyrate aminotransferase, mitochondrial; Catalyzes the conversion of gamma-aminobutyrate and L- beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine; Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family (500 aa) | |||
| ZNF692 | Zinc finger protein 692; May be involved in transcriptional regulation; Zinc fingers C2H2-type (524 aa) | |||
| PCCB | propionyl-CoA carboxylase beta subunit (559 aa) | |||
| ECHDC1 | Ethylmalonyl-CoA decarboxylase; Decarboxylases ethylmalonyl-CoA decarboxylase, a potentially toxic metabolite, to form butyryl-CoA, suggesting it might be involved in metabolite proofreading. Also has methylmalonyl-CoA decarboxylase activity at lower level (307 aa) | |||
| MDH1 | Malate dehydrogenase, cytoplasmic; Malate dehydrogenase 1; Belongs to the LDH/MDH superfamily. MDH type 2 family (352 aa) | |||
| ACSS3 | Acyl-CoA synthetase short-chain family member 3, mitochondrial; Activates acetate so that it can be used for lipid synthesis or for energy generation; Belongs to the ATP-dependent AMP-binding enzyme family (686 aa) | |||
| ALDH6A1 | Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial; Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA; Aldehyde dehydrogenases (535 aa) | |||
| HADH | Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial; Plays an essential role in the mitochondrial beta- oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA; Belongs to the 3-hydroxyacyl-CoA dehydrogenase family (331 aa) | |||
