Ontology highlight
ABSTRACT:
SUBMITTER: Lanson NA
PROVIDER: S-EPMC4288133 | biostudies-literature | 2011 Jul
REPOSITORIES: biostudies-literature
Lanson Nicholas A NA Maltare Astha A King Hanna H Smith Rebecca R Kim Ji Han JH Taylor J Paul JP Lloyd Thomas E TE Pandey Udai Bhan UB
Human molecular genetics 20110412 13
Amyotrophic lateral sclerosis (ALS) is a late-onset neurodegenerative disorder characterized by the loss of motor neurons. Fused in sarcoma/translated in liposarcoma (FUS/TLS) and TAR DNA-binding protein (TDP)-43 are DNA/RNA-binding proteins found to be mutated in sporadic and familial forms of ALS. Ectopic expression of human ALS-causing FUS/TLS mutations in Drosophila caused an accumulation of ubiquitinated proteins, neurodegeneration, larval-crawling defect and early lethality. Mutant FUS/TLS ...[more]