Multi-phaseted problems of TDP-43 in selective neuronal vulnerability in ALS

Abstract excerpt

Transactive response DNA-binding protein 43 kDa (TDP-43) encoded by the TARDBP gene is an evolutionarily conserved heterogeneous nuclear ribonucleoprotein (hnRNP) that regulates multiple steps of RNA metabolism, and its cytoplasmic aggregation characterizes degenerating motor neurons in amyotrophic lateral sclerosis (ALS). In most ALS cases, cytoplasmic TDP-43 aggregation occurs in the absence of mutations in the...

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• Amyotrophic Lateral Sclerosis Research
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• RNA Research and Splicing