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A case of cerebrotendinous xanthomatosis. II: The sterol content of a cataractous lens.
  1. P. McKenna,
  2. S. J. Morgan,
  3. R. C. Bosanquet and
  4. M. F. Laker
  1. Department of Clinical Biochemistry, Royal Victoria Infirmary, Newcastle upon Tyne.

    Abstract

    The cholestanol content of a cataractous lens nucleus from a patient with cerebrotendinous xanthomatosis (CTX) was quantified by gas chromatography-mass spectrometry and found to be 0.27 micrograms per mg freeze-dried lens tissue. The cholestanol-cholesterol ratio of 1.7% in the lens nucleus was similar to that in the serum of the CTX patient. The cholestanol content and cholestanol-cholesterol ratio in the CTX lens were approximately four-fold and six-fold greater respectively than the mean levels found in three senile cataractous lens nuclei analysed simultaneously for comparative purposes.

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