Cholestanol

Cholestanol is a cholesterol metabolite formed by oxidation and an intermediate in the biosynthesis of chenodeoxycholic acid (Item No. 10011286).¹ Cholestanol (10 µg/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

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1. Serizawa, S., Otsuka, H., Seyama, Y., et al. Studies on the biosynthesis of cholestanol in cultured cells. J. Biochem. 92(5), 1547-1557 (1982).

2. Inoue, K., Kubota, S., Tsuru, T., et al. Cholestanol induces apoptosis of corneal endothelial and lens epithelial cells. Invest. Ophthalmol. Vis. Sci. 41(5), 991-997 (2000).

3. Seyama, Y. Cholestanol metabolism, molecular pathology, and nutritional implications. J. Med. Food 6(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 xanthomatosis. Neurologia 26(7), 397-404 (2011).