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Aldh3a1 | Aldehyde dehydrogenase, dimeric NADP-preferring; ALDHs play a major role in the detoxification of alcohol- derived acetaldehyde (Probable). They are involved in the metabolism of corticosteroids, biogenic amines, neurotransmitters, and lipid peroxidation (Probable). Oxidizes medium and long chain aldehydes into non-toxic fatty acids. Preferentially oxidizes aromatic aldehyde substrates. Comprises about 50 percent of corneal epithelial soluble proteins. May play a role in preventing corneal damage caused by ultraviolet light. (453 aa) | ||||
Dpys | Dihydropyrimidinase; Catalyzes the second step of the reductive pyrimidine degradation, the reversible hydrolytic ring opening of dihydropyrimidines. Can catalyze the ring opening of 5,6-dihydrouracil to N-carbamyl-alanine and of 5,6-dihydrothymine to N-carbamyl-amino isobutyrate (By similarity). (519 aa) | ||||
Ehhadh | Enoyl-CoA hydratase/3,2-trans-enoyl-CoA isomerase; In the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase family. (718 aa) | ||||
Echs1 | Enoyl-CoA hydratase, mitochondrial; Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate (By similarity). Has high substrate specificity for crotonyl-CoA and moderate specificity for acryloyl-CoA, 3-methylcrotonyl-CoA and methacrylyl-CoA. It is noteworthy that binds tiglyl-CoA, but hydrates only a small amount of this substrate (By similarity). (290 aa) | ||||
Aldh9a1 | 4-trimethylaminobutyraldehyde dehydrogenase; Converts gamma-trimethylaminobutyraldehyde into gamma- butyrobetaine with high efficiency (in vitro). Can catalyze the irreversible oxidation of a broad range of aldehydes to the corresponding acids in an NAD-dependent reaction, but with low efficiency. (518 aa) | ||||
Gad2 | Glutamate decarboxylase 2; Catalyzes the production of GABA. (585 aa) | ||||
Aldh2 | Aldehyde dehydrogenase, mitochondrial; Is capable of converting retinaldehyde to retinoic acid. (519 aa) | ||||
Acads | Short-chain specific acyl-CoA dehydrogenase, mitochondrial; Short-chain specific acyl-CoA dehydrogenase is one of the acyl-CoA dehydrogenases that catalyze the first step of mitochondrial fatty acid beta-oxidation, an aerobic process breaking down fatty acids into acetyl-CoA and allowing the production of energy from fats. The first step of fatty acid beta-oxidation consists in the removal of one hydrogen from C-2 and C-3 of the straight-chain fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA. Among the different mitochondrial acyl-CoA dehydrogenases, short-chai [...] (412 aa) | ||||
Dpyd | Dihydropyrimidine dehydrogenase [NADP(+)]; Involved in pyrimidine base degradation. Catalyzes the reduction of uracil and thymine (By similarity). (1025 aa) | ||||
Aoc2 | Retina-specific copper amine oxidase; Has a monoamine oxidase activity with substrate specificity for 2-phenylethylamine and tryptamine. May play a role in adipogenesis. May be a critical modulator of signal transmission in retina (By similarity); Belongs to the copper/topaquinone oxidase family. (757 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 (By similarity). (519 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 (By similarity); Belongs to the enoyl-CoA hydratase/isomerase family. (385 aa) | ||||
Upb1 | Beta-ureidopropionase; Catalyzes a late step in pyrimidine degradation. Converts N- carbamoyl-beta-alanine (3-ureidopropanoate) into beta-alanine, ammonia and carbon dioxide. Likewise, converts N-carbamoyl-beta- aminoisobutyrate (3-ureidoisobutyrate) into beta-aminoisobutyrate, ammonia and carbon dioxide; Belongs to the carbon-nitrogen hydrolase superfamily. BUP family. (393 aa) | ||||
Aldh3b1 | Aldehyde dehydrogenase family 3 member B1; Oxidizes medium and long chain saturated and unsaturated aldehydes. Metabolizes also benzaldehyde (By similarity). Low activity towards acetaldehyde and 3,4- dihydroxyphenylacetaldehyde (By similarity). May not metabolize short chain aldehydes. Can use both NADP(+) and NAD(+) as electron acceptor (By similarity). May have a protective role against the cytotoxicity induced by lipid peroxidation (By similarity). (468 aa) | ||||
Acox1 | Peroxisomal acyl-CoA oxidase 1, A chain; Catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs (By similarity). Isoform 1 shows highest activity against medium-chain fatty acyl-CoAs and activity decreases with increasing chain length (By similarity). Isoform 2 is active against a much broader range of substrates and shows activity towards very long-chain acyl-CoAs (By similarity). (661 aa) | ||||
Acox3 | Peroxisomal acyl-coenzyme A oxidase 3; Oxidizes the CoA-esters of 2-methyl-branched fatty acids. (700 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 (By similarity). (500 aa) | ||||
Aldh7a1 | Alpha-aminoadipic semialdehyde dehydrogenase; Multifunctional enzyme mediating important protective effects. Metabolizes betaine aldehyde to betaine, an important cellular osmolyte and methyl donor. Protects cells from oxidative stress by metabolizing a number of lipid peroxidation-derived aldehydes. Involved in lysine catabolism (By similarity). (539 aa) | ||||
Cndp1 | Beta-Ala-His dipeptidase; Belongs to the peptidase M20A family. (492 aa) | ||||
Aldh3a2 | Aldehyde dehydrogenase family 3 member A2; Catalyzes the oxidation of medium and long-chain aliphatic aldehydes to fatty acids. Active on a variety of saturated and unsaturated aliphatic aldehydes between 6 and 24 carbons in length. Responsible for conversion of the sphingosine 1- phosphate (S1P) degradation product hexadecenal to hexadecenoic acid. (484 aa) | ||||
Gadl1 | Acidic amino acid decarboxylase GADL1; Catalyzes the decarboxylation of L-aspartate, 3-sulfino-L- alanine (cysteine sulfinic acid), and L-cysteate to beta-alanine, hypotaurine and taurine, respectively. The preferred substrate is L- aspartate. Does not exhibit any decarboxylation activity toward glutamate. (502 aa) | ||||
Aldh6a1 | Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial; Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA (By similarity). (535 aa) | ||||
Gad1 | Glutamate decarboxylase 1; Catalyzes the production of GABA; Belongs to the group II decarboxylase family. (593 aa) | ||||
Mlycd | Malonyl-CoA decarboxylase, mitochondrial; Catalyzes the conversion of malonyl-CoA to acetyl-CoA. In the fatty acid biosynthesis MCD selectively removes malonyl-CoA and thus assures that methyl-malonyl-CoA is the only chain elongating substrate for fatty acid synthase and that fatty acids with multiple methyl side chains are produced. In peroxisomes it may be involved in degrading intraperoxisomal malonyl-CoA, which is generated by the peroxisomal beta-oxidation of odd chain-length dicarboxylic fatty acids. Plays a role in the metabolic balance between glucose and lipid oxidation in mus [...] (492 aa) | ||||
Aoc3 | Membrane primary amine oxidase; Cell adhesion protein that participates in lymphocyte recirculation by mediating the binding of lymphocytes to peripheral lymph node vascular endothelial cells in an L-selectin-independent fashion. Has a monoamine oxidase activity. May play a role in adipogenesis (By similarity). (765 aa) | ||||
Aldh3b2 | Aldehyde dehydrogenase family 3 member B2; Oxidizes medium and long chain aldehydes into non-toxic fatty acids; Belongs to the aldehyde dehydrogenase family. (479 aa) | ||||
Hadha | Trifunctional enzyme subunit alpha, mitochondrial; Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway. The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA. Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids. Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional en [...] (763 aa) | ||||
Cndp2 | Cytosolic non-specific dipeptidase; Hydrolyzes a variety of dipeptides including L-carnosine but has a strong preference for Cys-Gly. Catalyzes the production of N- lactoyl-amino acids from lactate and amino acids by reverse proteolysis. (475 aa) | ||||
Carns1 | Carnosine synthase 1; Catalyzes the synthesis of carnosine and homocarnosine. Carnosine is synthesized more efficiently than homocarnosine. (947 aa) | ||||
Aldh3b3 | Aldehyde dehydrogenase family 3 member B3; Oxidizes medium and long chain aldehydes into non-toxic fatty acids. (479 aa) |