Article
Axon guidance genes modulate neurotoxicity of ALS-associated UBQLN2.
eLife - 11 Apr 2023
Kim Sang Hwa, Nichols Kye D, Anderson Eric N, Liu Yining, Ramesh Nandini, Jia Weiyan, Kuerbis Connor J, Scalf Mark, Smith Lloyd M, Pandey Udai Bhan, Tibbetts Randal S
Abstract excerpt
Mutations in the ubiquitin (Ub) chaperone Ubiquilin 2 (UBQLN2) cause X-linked forms of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) through unknown mechanisms. Here, we show that aggregation-prone, ALS-associated mutants of UBQLN2 (UBQLN2ALS) trigger heat stress-dependent neurodegeneration in Drosophila. A genetic modifier screen implicated endolysosomal and axon guidance genes, including...
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