Oxidative Imbalance in Nonstimulated X-Adrenoleukodystrophy-Derived Lymphoblasts
نویسندگان
چکیده
منابع مشابه
The X-linked adrenoleukodystrophy (X-ALD) and oxidative stress.
Most of the studies indicate that there is as yet no complete cure for X-ALD. However, methods of the treatment seem to slow rather than treat the disease. One method is the use of Lorenzo's oil in conjunction with a low fat diet, which may help in cerebral X-ALD. X-ALD is in very close resemblance to another neurodegenerative disease, amyotrophic lateral sclerosis (ALS). One of the believed pa...
متن کاملEarly oxidative damage underlying neurodegeneration in X-adrenoleukodystrophy.
X-linked adrenoleukodystrophy (X-ALD) is a fatal neurodegenerative disorder, characterized by progressive cerebral demyelination cerebral childhood adrenoleukodystrophy (CCALD) or spinal cord neurodegeneration (adrenomyeloneuropathy, AMN), adrenal insufficiency and accumulation of very long-chain fatty acids (VLCFA) in tissues. The disease is caused by mutations in the ABCD1 gene, which encodes...
متن کاملInflammation in X - linked Adrenoleukodystrophy
Project Description: Cells compute by processing external stimuli according to their internal state, abiding to rules that remain poorly understood (Rubens et al., 2016). Cell fate decisions are the key for constructing a multicellular organism. Errors, bias or aberrant delays in making such decisions can lead to neoplasia or in extreme cases, tumorigenesis. Our key question is how cells make d...
متن کاملMRI in X-linked adrenoleukodystrophy.
A 45-year-old man with no medical history presented with behavioral changes. He was withdrawn from his family and friends. Home and finances had fallen into disorder. He was disheveled and incontinent of urine and feces. He lacked insight into his cognitive decline. His skin was bronzed and the examination was notable only for upgoing plantar responses. Peroxisomal fatty acid profile was elevat...
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: Developmental Neuroscience
سال: 2008
ISSN: 1421-9859,0378-5866
DOI: 10.1159/000191212