Ontology highlight
ABSTRACT:
SUBMITTER: Coyne AN
PROVIDER: S-EPMC5007633 | biostudies-literature | 2015 Dec
REPOSITORIES: biostudies-literature
Coyne Alyssa N AN Yamada Shizuka B SB Siddegowda Bhavani Bagevalu BB Estes Patricia S PS Zaepfel Benjamin L BL Johannesmeyer Jeffrey S JS Lockwood Donovan B DB Pham Linh T LT Hart Michael P MP Cassel Joel A JA Freibaum Brian B Boehringer Ashley V AV Taylor J Paul JP Reitz Allen B AB Gitler Aaron D AD Zarnescu Daniela C DC
Human molecular genetics 20150918 24
RNA dysregulation is a newly recognized disease mechanism in amyotrophic lateral sclerosis (ALS). Here we identify Drosophila fragile X mental retardation protein (dFMRP) as a robust genetic modifier of TDP-43-dependent toxicity in a Drosophila model of ALS. We find that dFMRP overexpression (dFMRP OE) mitigates TDP-43 dependent locomotor defects and reduced lifespan in Drosophila. TDP-43 and FMRP form a complex in flies and human cells. In motor neurons, TDP-43 expression increases the associat ...[more]