TDP-43 in the pathogenesis of amyotrophic lateral sclerosis (ALS)
Vivek Swarup
Broschiertes Buch

TDP-43 in the pathogenesis of amyotrophic lateral sclerosis (ALS)

Study and characterization of TDP-43 mutants in the pathogenesis of ALS

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Amyotrophic lateral sclerosis (ALS) is a lethal disease characterized by degeneration of lower and upper motor neurons. Transactive response DNA-binding protein 43 (TDP-43) ubiquitinated inclusions are a hallmark of ALS. In order to understand the pathogenic mechanism caused by TDP-43, we generated transgenic mice using genomic fragments encoding human TDP-43 wild-type or FALS-linked mutants TDP-43G348C and TDP-43A315T and used these mice to study the pathogenic mechanism associated with ALS. These novel TDP-43 transgenic mice develop many age-related pathological and biochemical changes remin...