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肾上腺脑白质营养不良中髓磷脂的生化研究。

Biochemical study on myelin in adrenoleukodystrophy.

作者信息

Miyatake T, Ariga T, Atsumi T, Komiya Y

出版信息

Adv Exp Med Biol. 1978;100:585-99. doi: 10.1007/978-1-4684-2514-7_43.

DOI:10.1007/978-1-4684-2514-7_43
PMID:696481
Abstract

Two myelin fractions, heavy and light, were isolated from white matter of a patient with adrenoleukodystrophy. Morphologically, heavy myelin fraction showed compact lamellar structures, and light myelin fraction consisted of loose lamellar structures and rod-like clear space. Analytical studies demonstrated that the chemical composition of heavy myelin fraction was almost the same as that of normal myelin and that light myelin fraction consisted mainly of cholesterol. Long chain saturated fatty acids, C25:0 and C26:0, were increased and C24:1 was decreased in sphingoglycolipids of heavy myelin fraction of the patient.

摘要

从一名肾上腺脑白质营养不良患者的白质中分离出了两种髓磷脂组分,即重质组分和轻质组分。在形态学上,重质髓磷脂组分呈现紧密的层状结构,而轻质髓磷脂组分则由松散的层状结构和棒状透明间隙组成。分析研究表明,重质髓磷脂组分的化学成分与正常髓磷脂几乎相同,轻质髓磷脂组分主要由胆固醇组成。该患者重质髓磷脂组分的鞘糖脂中长链饱和脂肪酸C25:0和C26:0增加,C24:1减少。

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1
Biochemical study on myelin in adrenoleukodystrophy.肾上腺脑白质营养不良中髓磷脂的生化研究。
Adv Exp Med Biol. 1978;100:585-99. doi: 10.1007/978-1-4684-2514-7_43.
2
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2
Determination of C20-C30 fatty acids by reversed-phase chromatographic techniques: an efficient method to quantitate minor fatty acids in serum of patients with adrenoleukodystrophy.
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