Inherited and acquired disorders of myelin: The underlying myelin pathology
نویسندگان
چکیده
منابع مشابه
Cell transplantation strategies for acquired and inherited disorders of peripheral myelin
Objective To investigate transplantation of rat Schwann cells or human iPSC-derived neural crest cells and derivatives into models of acquired and inherited peripheral myelin damage. Methods Primary cultured rat Schwann cells labeled with a fluorescent protein for monitoring at various times after transplantation. Human-induced pluripotent stem cells (iPSCs) were differentiated into neural cr...
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15 صفحه اولRegion-specific myelin pathology in mice lacking the golli products of the myelin basic protein gene.
The myelin basic protein (MBP) gene encodes two families of proteins, the classic MBP constituents of myelin and the golli-MBPs, the function of which is less well understood. In this study, targeted ablation of the golli-MBPs, but not the classic MBPs, resulted in a distinct phenotype unlike that of knock-outs (KOs) of the classic MBPs or other myelin proteins. Although the golli KO animals di...
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Charcot-Marie-Tooth disease (CMT) is an inherited disorder of the peripheral nerves, which involves slowly progressive weakness and atrophy of the distal muscles (Charcot and Marie, 1886; Tooth, 1886). CMT is a type of hereditary motor and sensory neuropathy that exhibits both clinical and genetic heterogeneity (Dyck et al., 1993; Lupski et al., 1991a). Clinical features include initial weaknes...
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ژورنال
عنوان ژورنال: Experimental Neurology
سال: 2016
ISSN: 0014-4886
DOI: 10.1016/j.expneurol.2016.04.002