Article
Fragile X protein mitigates TDP-43 toxicity by remodeling RNA granules and restoring translation.
Human molecular genetics - 15 Dec 2015
Coyne Alyssa N, Yamada Shizuka B, Siddegowda Bhavani Bagevalu, Estes Patricia S, Zaepfel Benjamin L, Johannesmeyer Jeffrey S, Lockwood Donovan B, Pham Linh T, Hart Michael P, Cassel Joel A, Freibaum Brian, Boehringer Ashley V, Taylor J Paul, Reitz Allen B, Gitler Aaron D, Zarnescu Daniela C
Abstract excerpt
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...
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