5α-Cholestan-3β-ol (5α-Cholestanol)

Cholestanol is a cholesterol metabolite formed by oxidation and an intermediate in the biosynthesis of chenodeoxycholic acid .¹ Cholestanol (10 ?g/ml) induces apoptosis in cornea and lens epithelial cells and increases the activity of IL-1β converting enzyme (ICE) and CPP32 proteases.² Dietary administration of 1% cholestanol to mice increases serum and liver cholestanol levels and leads to corneal opacities and gallstones and in rats it leads to cholestanol deposition in the cerebellum.³ Cholestanol levels are increased in plasma of patients with cerebrotendinous xanthomatosis (CTX), a disease characterized by a deficiency in the mitochondrial enzyme sterol 27-hydrolylase (CYP27A1) that leads to progressive neurological symptoms.⁴

1.Serizawa, S., Otsuka, H., Seyama, Y., et al.Studies on the biosynthesis of cholestanol in cultured cellsJ. Biochem.92(5)1547-1557(1982) 2.Inoue, K., Kubota, S., Tsuru, T., et al.Cholestanol induces apoptosis of corneal endothelial and lens epithelial cellsInvest. Ophthalmol. Vis. Sci.41(5)991-997(2000) 3.Seyama, Y.Cholestanol metabolism, molecular pathology, and nutritional implicationsJ. Med. Food6(3)217-224(2003) 4.Pilo de la Fuente, B., Sobrido, M.J., Girós, M., et al.Usefulness of cholestanol levels in the diagnosis and follow-up of patients with cerebrotendinous xanthomatosisNeurologia26(7)397-404(2011)