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Article

Bruno-3 regulates sarcomere component expression and contributes to muscle phenotypes of myotonic dystrophy type 1.

Disease models & mechanisms - 21 May 2018

Picchio Lucie, Legagneux Vincent, Deschamps Stephane, Renaud Yoan, Chauveau Sabine, Paillard Luc, Jagla Krzysztof

Abstract excerpt

Steinert disease, or myotonic dystrophy type 1 (DM1), is a multisystemic disorder caused by toxic noncoding CUG repeat transcripts, leading to altered levels of two RNA binding factors, MBNL1 and CELF1. The contribution of CELF1 to DM1 phenotypes is controversial. Here, we show that the Drosophila CELF1 family member, Bru-3, contributes to pathogenic muscle defects observed in a Drosophila model of DM1. Bru-3...

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Source

Open in PubMed Open full text

Identifiers

PMID: 29716962
DOI: 10.1242/dmm.031849
PMCID: PMC5992612

Genes

CELF1
MBNL1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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