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Article

TDP-43 suppresses CGG repeat-induced neurotoxicity through interactions with HnRNP A2/B1.

Human molecular genetics - 1 Oct 2014

He Fang, Krans Amy, Freibaum Brian D, Taylor J Paul, Todd Peter K

Abstract excerpt

Nucleotide repeat expansions can elicit neurodegeneration as RNA by sequestering specific RNA-binding proteins, preventing them from performing their normal functions. Conversely, mutations in RNA-binding proteins can trigger neurodegeneration at least partly by altering RNA metabolism. In Fragile X-associated tremor/ataxia syndrome (FXTAS), a CGG repeat expansion in the 5'UTR of the fragile X gene (FMR1) leads...

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Identifiers

PMID: 24920338
DOI: 10.1093/hmg/ddu216
PMCID: PMC4159148

Genes

FMR1
FUS

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 3 Sept 2026.

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