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Article

Molecular Mechanisms Underlying TDP-43 Pathology in Cellular and Animal Models of ALS and FTLD.

International journal of molecular sciences - 29 Apr 2021

Wood Alistair, Gurfinkel Yuval, Polain Nicole, Lamont Wesley, Lyn Rea Sarah

Abstract excerpt

Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are neurodegenerative disorders that exist on a disease spectrum due to pathological, clinical and genetic overlap. In up to 97% of ALS cases and ~50% of FTLD cases, the primary pathological protein observed in affected tissues is TDP-43, which is hyperphosphorylated, ubiquitinated and cleaved. The TDP-43 is observed in aggregates...

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Source

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Identifiers

PMID: 33946763
DOI: 10.3390/ijms22094705
PMCID: PMC8125728

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