Adrenoleukodystrophy: Increased plasma content of saturated very long chain fatty acids
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چکیده
منابع مشابه
Adrenoleukodystrophy: increased plasma content of saturated very long chain fatty acids.
With a new method we measured the saturated very long chain fatty acids in the plasma of adrenoleukodystrophy (ALD) hemizygotes, ALD heterozygotes, and controls. ALD hemizygotes showed increased levels of hexacosanoate (C26 fatty acid) which represented 0.081 +/- 0.0066% (SEM) of total fatty acids, compared to 0.015 +/- 0.0032% in the controls. C25, C24, and C23 fatty acids were also increased,...
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Measurement of plasma very long chain fatty acids is widely recognised as a sensitive screening test for X-linked adrenoleukodystrophy (X-ALD). This test has particular importance because of the highly variable clinical expression of X-ALD. In this affected family the progressive childhood form of X-ALD was accompanied by "non-diagnostic" concentrations of plasma very long chain fatty acids. Th...
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X-linked adrenoleukodystrophy (X-ALD, OMIM 300100) is a severe inherited neurodegenerative disease, associated with the accumulation of very long-chain fatty acids (VLCFA). The recent unexpected observation that the accumulation of VLCFA in tissues of the Abcd1-deficient mouse model for X-ALD is not due to a deficiency in VLCFA degradation, led to the hypothesis that mitochondrial abnormalities...
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ژورنال
عنوان ژورنال: Neurology
سال: 1981
ISSN: 0028-3878,1526-632X
DOI: 10.1212/wnl.31.10.1241