Identification of (23S)-5beta-cholestane-3alpha, 7alpha, 12alpha, 23, 25-pentol in cerebrotendinous xanthomatosis.
The synthesis of (23R)- and (23S)-5beta-cholestane-3alpha, 7alpha, 12alpha, 23, 25-pentols is described. Norcholyl aldehyde was converted into the cholestanepentols by a Reformatsky reaction with ethyl bromoacetate followed by a Grignard reaction with methylmagnesium iodide. One of the synthetic pentols, the 23S epimer was identical with a bile alcohol isolated from patients with cerebrotendinous xanthomatosis.
D001927:Brain Diseases / Q000378:metabolism*; D002849:Chromatography, Gas; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D013058:Mass Spectrometry; D008968:Molecular Conformation; D009904:Optical Rotation; D013237:Stereoisomerism; D013261:Sterols / Q000138:chemical synthesis / Q000378:metabolism*; D013710:Tendons / Q000378:metabolism; D014973:Xanthomatosis / Q000378:metabolism*
D013261:Sterols