7,8-Didehydrodesmosterol
IUPAC 名称
2,15-dimethyl-14-(6-methylhept-5-en-2-yl)tetracyclo[8.7.0.0^{2,7}.0^{11,15}]heptadeca-7,9-dien-5-ol
描述
Constituent of pig tissues and bakers yeast (Saccharomyces cerevisiae)
7-Dehydrodesmosterol is a sterol intermediate in the biosynthesis of steroids. 7-Dehydrodesmosterol is a substrate of the enzyme 24-dehydrocholesterol reductase (EC:1.3.1.72), an important enzyme in the biosynthesis of Cholesterol. Cholesterol is synthesized from either Lathosterol, 7-Dehydrocholesterol, Desmosterol or Cholestenol by the enzyme 3beta-hydroxysterol delta7 reductase (EC 1.3.1.21, Dhcr7). The Smith-Lemli-Opitz syndrome (SLOS, OMIM 270400) is caused by a genetic defect in cholesterol biosynthesis; mutations in the enzyme 3beta-hydroxysterol delta7 reductase lead to a failure of cholesterol synthesis, with an accumulation of precursor sterols, such as 7-Dehydrodesmosterol. SLOS results in craniofacial, limb as well as major organ defects, including the brain. In individuals with this syndrome, mental retardation, as well as other CNS dysfunction, is almost 100% prevalent. (PMID: 15862627, 17197219). 7,8-Didehydrodesmosterol is found in animal foods.
异构
CC(CCC=C(C)C)C1CCC2C3=CC=C4CC(O)CCC4(C)C3CCC12C
InChI标识符
InChI=1S/C27H42O/c1-18(2)7-6-8-19(3)23-11-12-24-22-10-9-20-17-21(28)13-15-26(20,4)25(22)14-16-27(23,24)5/h7,9-10,19,21,23-25,28H,6,8,11-17H2,1-5H3
键
RUSSPKPUXDSHNC-UHFFFAOYSA-N