TDP-43 in central nervous system development and function: clues to TDP-43-associated neurodegeneration
نویسندگان
چکیده
منابع مشابه
Neurodegeneration: A Leg Up on TDP-43
TDP-43 is a key disease protein for amyotrophic lateral sclerosis but how it drives motor neuron degeneration remains unresolved. A new study has modeled TDP-43 age-dependent axonal death in the Drosophila leg and used a powerful forward genetic screen to identify three novel suppressor genes.
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TAR DNA-binding protein (TDP-43) is a highly conserved and essential DNA- and RNA-binding protein that controls gene expression through RNA processing, in particular, regulation of splicing. Intracellular aggregation of TDP-43 is a hallmark of amyotrophic lateral sclerosis and ubiquitin-positive frontotemporal lobar degeneration. This TDP-43 pathology is also present in other types of neurodege...
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The budding yeast Saccharomyces cerevisiae is an emerging tool for investigating the molecular pathways that underpin several human neurodegenerative disorders associated with protein misfolding. Amyotrophic lateral sclerosis (ALS) is a devastating adult onset neurodegenerative disease primarily affecting motor neurons. The protein TDP-43 has recently been demonstrated to play an important role...
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The majority of cases of frontotemporal lobar degeneration and amyotrophic lateral sclerosis are pathologically defined by the cleavage, cytoplasmic redistribution and aggregation of TAR DNA binding protein of 43 kDa (TDP-43). To examine the contribution of these potentially toxic mechanisms in vivo, we generated transgenic mice expressing human TDP-43 containing the familial amyotrophic latera...
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ژورنال
عنوان ژورنال: Biological Chemistry
سال: 2012
ISSN: 1437-4315,1431-6730
DOI: 10.1515/hsz-2012-0115