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Article

Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice

4 Jan 2011

Abstract excerpt

Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are characterized by cytoplasmic protein aggregates in the brain and spinal cord that include TAR-DNA binding protein 43 (TDP-43). TDP-43 is normally localized in the nucleus with roles in the regulation of gene expression, and pathological cytoplasmic aggregates are associated with depletion of nuclear protein. Here, we generated...

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PMID: 21206091
DOI: 10.1172/jci44867

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