[HTML][HTML] Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation
MJ Winton, LM Igaz, MM Wong, LK Kwong… - Journal of Biological …, 2008 - ASBMB
TAR DNA-binding protein 43 (TDP-43) is the disease protein in frontotemporal lobar
degeneration with ubiquitin-positive inclusions (FTLD-U) and amyotrophic lateral sclerosis
(ALS). Although normal TDP-43 is a nuclear protein, pathological TDP-43 is redistributed
and sequestered as insoluble aggregates in neuronal nuclei, perikarya, and neurites. Here
we recapitulate these pathological phenotypes in cultured cells by altering endogenous TDP-
43 nuclear trafficking and by expressing mutants with defective nuclear localization (TDP-43 …
degeneration with ubiquitin-positive inclusions (FTLD-U) and amyotrophic lateral sclerosis
(ALS). Although normal TDP-43 is a nuclear protein, pathological TDP-43 is redistributed
and sequestered as insoluble aggregates in neuronal nuclei, perikarya, and neurites. Here
we recapitulate these pathological phenotypes in cultured cells by altering endogenous TDP-
43 nuclear trafficking and by expressing mutants with defective nuclear localization (TDP-43 …