Evolution of adrenoleukodystrophy model systems
نویسندگان
چکیده
منابع مشابه
A mouse model for X-linked adrenoleukodystrophy.
X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disorder with impaired beta-oxidation of very long chain fatty acids (VLCFAs) and reduced function of peroxisomal very long chain fatty acyl-CoA synthetase (VLCS) that leads to severe and progressive neurological disability. The X-ALD gene, identified by positional cloning, encodes a peroxisomal membrane protein (adrenoleukodystrophy protei...
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15 صفحه اولAdrenoleukodystrophy: case report.
Adrenoleukodystrophy (ALD) is an X-linked neurodegenerative disorder associated with progressive central demyelination and adrenal insufficiency1. It was first described by Siemerling and Creutzfeldt2 in 1923 as “bronzed sclerosing encephalomyelitis. In 1970, Biaw assigned the now generally used term adrenoleukodystrophy. It is believed to be peroxisomal disease and biochemically characterized ...
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A generalized simplified model for describing the dynamic behavior of distributed parameter systems is proposed. The various specific characteristics of gain and phase angle of distributed parameter systems are investigated from frequency response formulation and complex plane representation of the proposed simplified model. The complex plane investigation renders some important inequality cons...
متن کاملPathophysiology of X-linked adrenoleukodystrophy☆
Currently the molecular basis for the clinical heterogeneity of X-linked adrenoleukodystrophy (X-ALD) is poorly understood. The genetic bases for all different phenotypic variants of X-ALD are mutations in the gene encoding the peroxisomal ATP-binding cassette (ABC) transporter, ABCD1 (formerly adrenoleukodystrophy protein, ALDP). ABCD1 transports CoA-activated very long-chain fatty acids from ...
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ژورنال
عنوان ژورنال: Journal of Inherited Metabolic Disease
سال: 2021
ISSN: 0141-8955,1573-2665
DOI: 10.1002/jimd.12357