Article

ALS-FUS mutation affects the activities of HuD/ELAVL4 and FMRP leading to axon phenotypes in motoneurons

2020-08-26

Abstract excerpt

<h4>ABSTRACT</h4> Mutations in the RNA-binding protein (RBPs) FUS have been genetically associated with the motoneuron disease amyotrophic lateral sclerosis (ALS). Using both human induced pluripotent stem cells and mouse models, we found that FUS-ALS causative mutations affect the activity of two relevant RBPs with important roles in neuronal RNA metabolism: HuD/ELAVL4 and FMRP. Mechanistically, mutant FUS leads...

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Literature Corpus work: 6823d68b-7686-532b-a729-d9b54c9385d5
DOI: 10.1101/2020.08.26.268631

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