NDLI: Accumulation of very long chain fatty acids is common to 3 variants of adrenoleukodystrophy (ALD): “Classical” ALD, atypical ALD (female patient) and adrenomyeloneuropathy

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Accumulation of very long chain fatty acids is common to 3 variants of adrenoleukodystrophy (ALD): “Classical” ALD, atypical ALD (female patient) and adrenomyeloneuropathy

Content Provider	Semantic Scholar
Author	Molzer, Brunhilde Bernheimer, Hanno Budka, H. Pilz, Peter K. D. Toifl, Karl
Copyright Year	1981
Abstract	Fatty acids of cholesterol esters were analyzed by gas chromatography in affected CNS white matter of 3 variants of ALD ("classical" ALD, atypical ALD (adult female) and AMN) and of 10 controls with myelin breakdown of an etiology other than ALD. In all 3 ALD variants a marked accumulation of very long chain fatty acids (VLFA) as compared to control material was observed. This was due to the accumulation mainly of saturated C24-C32 fatty acids, particularly of C26:0, C25:0 and, to a lesser extent, C24:0 and C27:0 fatty acids. Our results demonstrate for the first time an accumulation of VLFA in an adult female patient (atypical ALD), who probably is an ALD heterozygote rather than a variant of AMN, and confirm and extend earlier findings in classical ALD and AMN, respectively. It appears that ALD may be a single nosological entity with clinically and morphologically different variants sharing specific ultrastructural (accumulation of paired leaflets) and neuro-biochemical (accumulation of VLFA) diagnostic markers.
Starting Page	301
Ending Page	310
Page Count	10
File Format	PDF HTM / HTML
DOI	10.1016/0022-510X(81)90108-8
PubMed reference number	7276981
Journal	Medline
Volume Number	51
Alternate Webpage(s)	https://api.elsevier.com/content/article/pii/0022510X81901088
Alternate Webpage(s)	https://www.sciencedirect.com/science/article/pii/0022510X81901088?dgcid=api_sd_search-api-endpoint
Alternate Webpage(s)	https://doi.org/10.1016/0022-510X%2881%2990108-8
Journal	Journal of the Neurological Sciences
Language	English
Access Restriction	Open
Content Type	Text
Resource Type	Article

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