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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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Aging
Central Nervous System
Disease Models, Animal
Mice, Inbred C57BL

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Mice, Transgenic

Muscular Atrophy

Neurodegenerative Diseases

Spatio-Temporal Analysis

Source

Identifiers

PMID: 29577934
DOI: 10.1016/j.ajpath.2018.03.002

Genes

TARDBP

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Selective Spatiotemporal Vulnerability of Central Nervous System Neurons to Pathologic TAR DNA-Binding Protein 43 in Aged Transgenic Mice.PMID 29577934Read ↗

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