Protein aggregation in amyotrophic lateral sclerosis
نویسندگان
چکیده
منابع مشابه
Mutant Profilin1 Aggregation in Amyotrophic Lateral Sclerosis: An in Vivo Biochemical Analysis
Introduction: Profilin1 (PFN1) is a ubiquitously expressed protein known for its function as a regulator of actin polymerization and dynamics. A recent discovery linked mutant PFN1 to Amyotrophic Lateral Sclerosis (ALS), which is a fatal and progressive motor neuron disease. We have also demonstrated that Gly118Val mutation in PFN1 is a cause of ALS, and the formation of aggregates containing m...
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Amyotrophic lateral sclerosis is a rapidly progressing fatal neurodegenerative disease characterized by the presence of protein inclusions within affected motor neurons. Endoplasmic reticulum stress leading to apoptosis was recently recognized to be an important process in the pathogenesis of sporadic human amyotrophic lateral sclerosis as well as in transgenic models of mutant superoxide dismu...
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Behçet’s disease is a multisystem vasculitis. Its neurological involvement mostly includes parenchymal and non-parenchymal central nervous system manifestations. Peripheral nervous system presentations are rare. A 32-yr-old male patient who fulfilled the international study group criteria for Behçet’s disease, referred to our center with walking difficulty and repeated falling downs. Neurologi...
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The nature of the "toxic gain of function" that results from amyotrophic lateral sclerosis (ALS)-, Parkinson-, and Alzheimer-related mutations is a matter of debate. As a result no adequate model of any neurodegenerative disease etiology exists. We demonstrate that two synergistic properties, namely, increased protein aggregation propensity (increased likelihood that an unfolded protein will ag...
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Aberrant accumulation of protein aggregates is a pathological hallmark of many neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS). Although a buildup of protein aggregates frequently leads to cell death, whether it is the key pathogenic factor in driving neurodegenerative disease remains controversial. HDAC6, a cytosolic ubiquitin-binding deacetylase, has emerged as an im...
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ژورنال
عنوان ژورنال: Acta Neuropathologica
سال: 2013
ISSN: 0001-6322,1432-0533
DOI: 10.1007/s00401-013-1125-6