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HSD17B10 | hydroxysteroid (17-beta) dehydrogenase 10; Functions in mitochondrial tRNA maturation. Part of mitochondrial ribonuclease P, an enzyme composed of MRPP1/TRMT10C, MRPP2/HSD17B10 and MRPP3/KIAA0391, which cleaves tRNA molecules in their 5’-ends. By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD) (261 aa) | |||
OXCT1 | 3-oxoacid CoA transferase 1; Key enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate (By similarity) (520 aa) | |||
AGXT2 | alanine--glyoxylate aminotransferase 2; 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 (514 aa) | |||
ACSS2 | acyl-CoA synthetase short-chain family member 2; Activates acetate so that it can be used for lipid synthesis or for energy generation (714 aa) | |||
HIBADH | 3-hydroxyisobutyrate dehydrogenase (336 aa) | |||
ABAT | 4-aminobutyrate aminotransferase; 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 (500 aa) | |||
ACSS1 | acyl-CoA synthetase short-chain family member 1 (689 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) | |||
AACS | acetoacetyl-CoA synthetase (672 aa) | |||
ALDH3A2 | aldehyde dehydrogenase 3 family, member A2; Catalyzes the oxidation of 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 (508 aa) | |||
ALDH9A1 | aldehyde dehydrogenase 9 family, member A1; Converts gamma-trimethylaminobutyraldehyde into gamma- butyrobetaine. Catalyzes the irreversible oxidation of a broad range of aldehydes to the corresponding acids in an NAD-dependent reaction (518 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) | |||
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) (482 aa) | |||
SCP2 | sterol carrier protein 2; Mediates in vitro the transfer of all common phospholipids, cholesterol and gangliosides between membranes. May play a role in regulating steroidogenesis (547 aa) | |||
OXCT2 | 3-oxoacid CoA transferase 2; Key enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate (By similarity) (517 aa) | |||
AOX1 | aldehyde oxidase 1 (1338 aa) | |||
PCCA | propionyl CoA carboxylase, alpha polypeptide (728 aa) | |||
ALDH1B1 | aldehyde dehydrogenase 1 family, member B1; 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) | |||
HADH | hydroxyacyl-CoA dehydrogenase; Plays an essential role in the mitochondrial beta- oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA (331 aa) | |||
ALDH7A1 | aldehyde dehydrogenase 7 family, member A1; 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 (539 aa) | |||
ACADM | acyl-CoA dehydrogenase, C-4 to C-12 straight chain; This enzyme is specific for acyl chain lengths of 4 to 16 (425 aa) | |||
PCCB | propionyl CoA carboxylase, beta polypeptide (559 aa) | |||
ACSS3 | acyl-CoA synthetase short-chain family member 3; Activates acetate so that it can be used for lipid synthesis or for energy generation (By similarity) (686 aa) | |||
ENSG00000257767 | Uncharacterized protein (245 aa) | |||
ALDH6A1 | aldehyde dehydrogenase 6 family, member A1; Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA (535 aa) |