[HTML][HTML] Ubiquilin modifies TDP-43 toxicity in a Drosophila model of amyotrophic lateral sclerosis (ALS)
KA Hanson, SH Kim, DA Wassarman… - Journal of Biological …, 2010 - ASBMB
TDP-43 (43-kDa TAR DNA-binding protein) is a major constituent of ubiquitin-positive
cytosolic aggregates present in neurons of patients with amyotrophic lateral sclerosis (ALS)
and ubiquitin-positive fronto-temporal lobar degeneration (FTLD-U). Inherited mutations in
TDP-43 have been linked to familial forms of ALS, indicating a key role for TDP-43 in
disease pathogenesis. Here, we describe a Drosophila melanogaster model of TDP-43
proteinopathy. Expression of wild-type human TDP-43 protein in Drosophila motor neurons …
cytosolic aggregates present in neurons of patients with amyotrophic lateral sclerosis (ALS)
and ubiquitin-positive fronto-temporal lobar degeneration (FTLD-U). Inherited mutations in
TDP-43 have been linked to familial forms of ALS, indicating a key role for TDP-43 in
disease pathogenesis. Here, we describe a Drosophila melanogaster model of TDP-43
proteinopathy. Expression of wild-type human TDP-43 protein in Drosophila motor neurons …
