Given the fact that NMJ pathology is considered an early event in ALS pathogenesis60 and that hnRNP R plays a role in the pathogenesis of neurodegenerative diseases such as frontotemporal dementia and ALS61–63, our study highlights the importance of local translation for maintaining the integrity of the neuromuscular system and points towards the possibility that hnRNP R dysfunction might contribute to the pathology underlying certain forms of neurodegenerative disorders. This evidence concerns the gene HNRNPR and frontotemporal dementia.