الصفحة الرئيسية>>Signaling Pathways>> Proteases>> Endogenous Metabolite>>8-dehydro Cholesterol

8-dehydro Cholesterol

رقم الكتالوجGC18544

يعد تركيز 8-dehydro Cholesterol المرتفع أحد السمات الكيميائية الحيوية التشخيصية لمتلازمة Smith-Lemli-Opitz الكلاسيكية (SLOS).

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8-dehydro Cholesterol التركيب الكيميائي

Cas No.: 70741-38-7

الحجم السعر المخزون الكميّة
1mg
71٫00
متوفر
5mg
317٫00
متوفر
10mg
561٫00
متوفر

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Sample solution is provided at 25 µL, 10mM.

Description Chemical Properties Product Documents Related Products

8-dehydro Cholesterol (8-DHC) is an isomer of the cholesterol precursor 7-DHC .[1][2][3] It accumulates in tissues and plasma in patients with Smith-Lemli-Opitz syndrome (SLOS), a disorder characterized by a mutation in DHCR7, the gene encoding 3β-hydroxysterol-Δ7-reductase, and decreased cholesterol levels in bodily tissues and fluids, as well as microcephaly, intellectual disability, and distinctive dysmorphic features. 8-DHC levels are increased in serum in a rat model of SLOS induced by the DHCR7 inhibitor AY 9944 .[1][2][3]

Reference:
[1]. Wolf, C., Chevy, F., Pham, J., et al. Changes in serum sterols of rats treated with 7-dehydrocholesterol-Δ7-reductase inhibitors: Comparison to levels in humans with Smith-Lemli-Opitz syndrome. J. Lipid. Res. 37(6), 1325-1333 (1996).
[2]. Björkhem, I., Starck, L., Andersson, U., et al. Oxysterols in the circulation of patients with the Smith-Lemli-Opitz syndrome: Abnormal levels of 24S- and 27-hydroxycholesterol. J. Lipid Res. 42(3), 366-371 (2001).
[3]. Griffiths, W.J., Abdel-Khalik, J., Crick, P.J., et al. Sterols and oxysterols in plasma from Smith-Lemli-Opitz syndrome patients. J. Steroid Biochem. Mol. Biol. 169, 77-87 (2017).

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