A dual role for 7-dehydrocholesterol reductase in regulating Hedgehog signalling?
نویسندگان
چکیده
منابع مشابه
Negative regulation of Hedgehog signaling by the cholesterogenic enzyme 7-dehydrocholesterol reductase.
Cholesterol regulates Hedgehog (Hh) signaling during early vertebrate development. Smith-Lemli-Opitz syndrome (SLOS) is caused by defects in 7-dehydrocholesterol reductase (DHCR7), an enzyme catalyzing the final step of cholesterol biosynthesis. Many developmental malformations attributed to SLOS occur in tissues and organs where Hh signaling is required for development, but the precise role of...
متن کاملReduced cholesterol levels impair Smoothened activation in Smith-Lemli-Opitz syndrome.
Smith-Lemli-Opitz syndrome (SLOS) is a common autosomal-recessive disorder that results from mutations in the gene encoding the cholesterol biosynthetic enzyme 7-dehydrocholesterol reductase (DHCR7). Impaired DHCR7 function is associated with a spectrum of congenital malformations, intellectual impairment, epileptiform activity and autism spectrum disorder. Biochemically, there is a deficit in ...
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Recently we identified GANT61, a small-molecule antagonist of Gli transcription factors, which are the final effectors of the mammalian Hedgehog (HH) signaling pathway. Here we describe a diamine substructure of GANT61 that carries the biological activity and show that this part of the molecule is structurally related to trans-1,4-bis(2-chlorobenzaminomethyl)cyclohexane dihydrochloride (AY9944)...
متن کاملEndogenous B-ring oxysterols inhibit the Hedgehog component Smoothened in a manner distinct from cyclopamine or side-chain oxysterols.
Cellular lipids are speculated to act as key intermediates in Hedgehog signal transduction, but their precise identity and function remain enigmatic. In an effort to identify such lipids, we pursued a Hedgehog pathway inhibitory activity that is particularly abundant in flagellar lipids of Chlamydomonas reinhardtii, resulting in the purification and identification of ergosterol endoperoxide, a ...
متن کاملThe RSH/"Smith-Lemli-Opitz" syndrome: historical footnote.
Thirty years after its clinical delineation in humans and its teratologic simulation in rats, a Garrodian error of metabolism was discovered in the autosomal recessive RSH/SLO syndrome, namely defective conversion of 7-dehydrocholesterol to cholesterol due to the mutant 7-dehydrocholesterol reductase. This opened the door to the study of several other defects of sterol biosynthesis in humans an...
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ورودعنوان ژورنال:
- Development
دوره 133 20 شماره
صفحات -
تاریخ انتشار 2006