Accession NumberHMDB02057
Common_NamePristanoyl-CoA
Description(R) Pristanoyl-CoA is converted by alpha-methylacyl-CoA racemase (E.C. 5.1.99.4) (S) pristanoyl-CoA, which is then degraded via peroxisomal beta-oxidation. Deficiency in this enzyme results in neuropathy, hypogonadism of adult onset; and in infant, defective bile acid synthesis has been observed. Pristanoyl-CoA is the substrate of propionyl-CoA C(2)-trimethyltridecanoyltransferase (E.C.2.3.1.154). It is the substrate of peroxisomal pristanoyl-CoA oxidase (E.C.1.3.3.6). A genetic disorder called Zellweger syndrome (OMIM: 214100), also known as neonatal adrenoleukodystrophy, NALD) is the result of a lack of pristanoyl-CoA oxidase, and the subsequent accumulation of phytanic acid and pristanic acid.
Chemical_IUPAC_Name[(2R,3R,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-[[hydroxy-[hydroxy-[3-hydroxy-2,2-dimethyl-3-[2-[2-(2,6,10,14-tetramethyl-3-oxo-pentadecanoyl)sulfanylethylcarbamoyl]ethylcarbamoyl]propoxy]phosphoryl]oxy-phosphoryl]oxymethyl]oxolan-3-yl]oxyphosphonic acid
Chemical FormulaC40H70N7O18P3S
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