脑腱黄瘤病一例。II：白内障晶状体的固醇含量
A case of cerebrotendinous xanthomatosis. II: The sterol content of a cataractous lens.
作者信息
McKenna P, Morgan S J, Bosanquet R C, Laker M F
机构信息
Department of Clinical Biochemistry, Royal Victoria Infirmary, Newcastle upon Tyne.
出版信息
Br J Ophthalmol. 1990 Oct;74(10):629-30. doi: 10.1136/bjo.74.10.629.
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.
摘要
采用气相色谱-质谱联用法定量分析了一名患有脑腱性黄瘤病（CTX）患者的白内障晶状体核中的胆甾烷醇含量，结果发现为每毫克冻干晶状体组织0.27微克。晶状体核中1.7%的胆甾烷醇-胆固醇比率与该CTX患者血清中的比率相似。为作比较同时分析了三个老年性白内障晶状体核，CTX晶状体中的胆甾烷醇含量和胆甾烷醇-胆固醇比率分别比其平均水平大约高四倍和六倍。
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