The Pathobiology of Amyotrophic Lateral Sclerosis: A Proteinopathy?
نویسندگان
چکیده
منابع مشابه
Mitochondrial pathobiology in Parkinson's disease and amyotrophic lateral sclerosis.
Parkinson's disease (PD) and amyotrophic lateral sclerosis (ALS) are the second and third most common human adult-onset neurodegenerative diseases, respectively, after Alzheimer's disease. They are characterized by prominent age-related neurodegeneration in selectively vulnerable neural systems. Some forms of PD and ALS are inherited, and genes causing these diseases have been identified. Morph...
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H erein, we review advances in understanding a group of disorders collectively known as TAR-DNA binding protein 43 (TDP-43) proteinopathies since the report that TDP-43 is the major disease protein that mechanistically links frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) with and without motor neuron disease to amyotrophic lateral sclerosis. Because TDP-43 protein...
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ژورنال
عنوان ژورنال: Journal of Neuropathology and Experimental Neurology
سال: 2005
ISSN: 0022-3069
DOI: 10.1097/01.jnen.0000173889.71434.ea