From Charcot to SOD1
نویسندگان
چکیده
منابع مشابه
From Charcot to SOD1 Mechanisms of Selective Motor Neuron Death in ALS
(Reaume et al., 1996). Further, some mutants such as SOD1 retain full specific activity (Borchelt et al., The sixth anniversary has just passed since the land1994), while neither the age of onset nor rapidity of mark discovery (Rosen et al., 1993) that mutation in the progression of human disease correlate with dismutase enzyme superoxide dismutase 1 (SOD1) is a primary activity levels (Cudkowi...
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Cu/Zn superoxide dismutase (SOD1) is an abundant enzyme that has been best studied as a regulator of antioxidant defense. Using the yeast Saccharomyces cerevisiae, we report that SOD1 transmits signals from oxygen and glucose to repress respiration. The mechanism involves SOD1-mediated stabilization of two casein kinase 1-gamma (CK1γ) homologs, Yck1p and Yck2p, required for respiratory repressi...
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Charcot-Marie-Tooth (CMT) disease is a well-known neural or spinal type of muscular atrophy. It is the most familiar disease within a group of conditions called Hereditary Motor and Sensory Neuropathies (HMSN). The disease was discovered by three scientists several years ago. Several genes are involved as the causative agents for the disease. Hundreds of causative mutations have been found and ...
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15 صفحه اولAggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1.
Analysis of transgenic mice expressing familial amyotrophic lateral sclerosis (ALS)-linked mutations in the enzyme superoxide dismutase (SOD1) have shown that motor neuron death arises from a mutant-mediated toxic property or properties. In testing the disease mechanism, both elimination and elevation of wild-type SOD1 were found to have no effect on mutant-mediated disease, which demonstrates ...
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ژورنال
عنوان ژورنال: Neuron
سال: 1999
ISSN: 0896-6273
DOI: 10.1016/s0896-6273(00)81108-3