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Article

Poly-dipeptides produced from C9orf72 hexanucleotide repeats cause selective motor neuron hyperexcitability in ALS.

Proceedings of the National Academy of Sciences of the United States of America - 15 Mar 2022

Jo Yunhee, Lee Jiwon, Lee Seul-Yi, Kwon Ilmin, Cho Hana

Abstract excerpt

SignificanceThe GGGGCC hexanucleotide repeat expansion in the chromosome 9 open reading frame 72 (C9orf72) gene is the most common genetic cause of amyotrophic lateral sclerosis (ALS). Despite myriad studies on the toxic effects of poly-dipeptides produced from the C9orf72 repeats, the mechanisms underlying the selective hyperexcitability of motor cortex that characterizes the early stages of C9orf72 ALS patients...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 35259014
DOI: 10.1073/pnas.2113813119
PMCID: PMC8931230

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