Brain endothelial dysfunction in cerebral adrenoleukodystrophy
نویسندگان
چکیده
منابع مشابه
Brain endothelial dysfunction in cerebral adrenoleukodystrophy.
See Aubourg (doi:10.1093/awv271) for a scientific commentary on this article.X-linked adrenoleukodystrophy is caused by mutations in the ABCD1 gene leading to accumulation of very long chain fatty acids. Its most severe neurological manifestation is cerebral adrenoleukodystrophy. Here we demonstrate that progressive inflammatory demyelination in cerebral adrenoleukodystrophy coincides with bloo...
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X-linked adrenoleukodystrophy (X-ALD) is an inherited neurodegenerative disease that affects approximately 1 in 25 000 males. It is characterized by elevated levels of saturated very long chain fatty acids (VLCFA), i.e., >C22:0, particularly in ganglioside and cholesterol ester fractions of brain white matter and adrenal cortex. Failure of peroxisomal very long chain fatty acyl-CoA synthetase (...
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ژورنال
عنوان ژورنال: Brain
سال: 2015
ISSN: 0006-8950,1460-2156
DOI: 10.1093/brain/awv250