Article
Selective Spatiotemporal Vulnerability of Central Nervous System Neurons to Pathologic TAR DNA-Binding Protein 43 in Aged Transgenic Mice.
The American journal of pathology - 1 Jun 2018
van Hummel Annika, Chan Gabriella, van der Hoven Julia, Morsch Marco, Ippati Stefania, Suh Lisa, Bi Mian, Asih Prita R, Lee Wei S, Butler Troy A, Przybyla Magdalena, Halliday Glenda M, Piguet Olivier, Kiernan Matthew C, Chung Roger S, Ittner Lars M, Ke Yazi D
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a rapidly progressing and fatal disease characterized by muscular atrophy because of loss of upper and lower motor neurons. Histopathologically, most patients with ALS have abnormal cytoplasmic accumulation and aggregation of the nuclear RNA-regulating protein TAR DNA-binding protein 43 (TDP-43). Pathogenic mutations in the TARDBP gene that encode TDP-43 have been identified...
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