IUPAC 名称
{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({[hydroxy(3-hydroxy-2,2-dimethyl-3-{[2-({2-[(2,6,10,14-tetramethyl-3-oxopentadecanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy)phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid
描述
(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. [HMDB]
InChI标识符
InChI=1S/C40H70N7O18P3S/c1-24(2)10-8-11-25(3)12-9-13-26(4)14-15-28(48)27(5)39(53)69-19-18-42-30(49)16-17-43-37(52)34(51)40(6,7)21-62-68(59,60)65-67(57,58)61-20-29-33(64-66(54,55)56)32(50)38(63-29)47-23-46-31-35(41)44-22-45-36(31)47/h22-27,29,32-34,38,50-51H,8-21H2,1-7H3,(H,42,49)(H,43,52)(H,57,58)(H,59,60)(H2,41,44,45)(H2,54,55,56)/t25?,26?,27?,29-,32-,33-,34?,38-/m1/s1