Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Progressive cardiac phenotypes and reduced reversibility from long-term CUGexp RNA expression in a DM1 mouse model.

JCI insight - 8 May 2026

Hu Rong-Chi, Tabary Mohammadreza, Wehrens Xander Ht, Cooper Thomas A

Abstract excerpt

Myotonic dystrophy type 1 (DM1) is caused by an expanded CTG repeat in the DMPK gene, resulting in mutant transcripts that form expanded CUG (CUGexp) RNA foci and sequester muscleblind-like (MBNL) RNA-binding proteins. DM1 is multisystemic, with progressive worsening of disease manifestations in affected tissues. Disease progression is attributed to somatic expansion of the CTG repeats with age, resulting in...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Disease Models, Animal
Share to Disease Progression

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Mice, Transgenic

Share to Myocardium

Share to Myotonic Dystrophy

Share to Myotonin-Protein Kinase

Share to RNA-Binding Proteins

Share to Trinucleotide Repeat Expansion

Source

Open in PubMed Open full text

Identifiers

PMID: 41855125
DOI: 10.1172/jci.insight.204278
PMCID: PMC13232024

Genes

DMPK

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Progressive cardiac phenotypes and reduced reversibility from long-term CUGexp RNA expression in a DM1 mouse model.PMID 41855125Read ↗

MBNL overexpression rescues cardiac phenotypes in a myotonic dystrophy type 1 heart mouse model.Semantic proximity Read ↗

RNA Interference Targeting CUG Repeats in a Mouse Model of Myotonic DystrophySemantic proximity Read ↗

Co-opting MBNL-dependent alternative splicing cassette exons to control gene therapy in myotonic dystrophySemantic proximity Read ↗

Transcriptional changes and developmental abnormalities in a zebrafish model of myotonic dystrophy type 1.Semantic proximity Read ↗

The sustained expression of Cas9 targeting toxic RNAs reverses disease phenotypes in mouse models of myotonic dystrophy type 1.Semantic proximity Read ↗

Expanded CCUG repeat RNA expression in Drosophila heart and muscle trigger Myotonic Dystrophy type 1-like phenotypes and activate autophagocytosis genesSemantic proximity Read ↗

Mice transgenic for the human myotonic dystrophy region with expanded CTG repeats display muscular and brain abnormalities.Semantic proximity Read ↗

Age of onset of RNA toxicity influences phenotypic severity: evidence from an inducible mouse model of myotonic dystrophy (DM1).Semantic proximity Read ↗

Mice lacking MBNL1 and MBNL2 exhibit sudden cardiac death and molecular signatures recapitulating myotonic dystrophySemantic proximity Read ↗

Targeting nuclear RNA for in vivo correction of myotonic dystrophy.Semantic proximity Read ↗

Myotonic dystrophy in transgenic mice expressing an expanded CUG repeat.Semantic proximity Read ↗

Systemic therapy in an RNA toxicity mouse model with an antisense oligonucleotide therapy targeting a non-CUG sequence within the DMPK 3′UTR RNASemantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.