Quantitative determination of cholestanol in plasma with mass fragmentography. Biochemical diagnosis of cerebrotendinous xanthomatosis.
A simple, sensitive, and accurate method for determination of cholestanol in plasma is described. In this method a fixed amount of cholestane is added to 1 ml of plasma as an internal standard and steroids are saponified and extracted with n-hexane. The amounts of cholestanol and cholesterol are determined by mass fragmentography by monitoring the intensities of m/e 306, m/e 329, and m/e 372 fragment ions. The relative standard deviation of results for cholestanol was about 6.3%. The cholestanol concentrations in the plasma, erythrocyte stroma, and plasma lipoproteins of three patients with cerebrotendinous xanthomatosis were determined by this method.
D000328:Adult; D004083:Cholestanol / Q000097:blood*; D002784:Cholesterol / Q000031:analogs & derivatives* / Q000097:blood; D005260:Female; D008401:Gas Chromatography-Mass Spectrometry / Q000379:methods; D006801:Humans; D008074:Lipoproteins / Q000097:blood; D008297:Male; D008832:Microchemistry; D014973:Xanthomatosis / Q000097:blood / Q000175:diagnosis*
D008074:Lipoproteins; D004083:Cholestanol; D002784:Cholesterol