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Biochemistry in demyclination. Tadashi MIYATAKE 1 , Shuzo SATO 1 , Takashi INUZUKA 1 1Department of Neurology, Brain Research Institute, Niigata University pp.1112-1121
Published Date 1986/12/10
DOI https://doi.org/10.11477/mf.1431905855
  • Abstract
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(1) Adrenoleukodystrophy (ALD)

 ALD is a fatal x-linked hereditary demyelinating disorder with the involvement of adrenal cortex. The characteristic biochemical abnormality of ALD is the increase of very long saturated fatty acids (VLCFA) in cholesterol esters, sphingolipids and glycerophospholipids and the disturbance of VLCFA metabolism was supposed to be the primary metabolic defect in this disorder. Singh et al. reported that the oxidation of VLCFA was impaired and peroxisomal very long chain fatty acyl-CoA synthetase was defective in ALD and Tsuji et al. found that the activities of microsomal fatty acid elongation were also increased in fibroblasts from patients with ALD.


Copyright © 1986, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1243 印刷版ISSN 0001-8724 医学書院

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