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Article

RNA Foci, CUGBP1, and ZNF9 are the primary targets of the mutant CUG and CCUG repeats expanded in myotonic dystrophies type 1 and type 2.

The American journal of pathology - 1 Nov 2011

Jones Karlie, Jin Bingwen, Iakova Polina, Huichalaf Claudia, Sarkar Partha, Schneider-Gold Christiane, Schoser Benedikt, Meola Giovanni, Shyu Ann-Bin, Timchenko Nikolai, Timchenko Lubov

Abstract excerpt

Expansions of noncoding CUG and CCUG repeats in myotonic dystrophies type 1 (DM1) and DM2 cause complex molecular pathology, the features of which include accumulation of RNA aggregates and misregulation of the RNA-binding proteins muscleblind-like 1 (MBNL1) and CUG-binding protein 1 (CUGBP1). CCUG repeats also decrease amounts of the nucleic acid binding protein ZNF9. Using tetracycline (Tet)-regulated...

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Identifiers

PMID: 21889481
DOI: 10.1016/j.ajpath.2011.07.013
PMCID: PMC3204036

Genes

MBNL1

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Related research

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Research trail→

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RNA Foci, CUGBP1, and ZNF9 are the primary targets of the mutant CUG and CCUG repeats expanded in myotonic dystrophies type 1 and type 2.PMID 21889481Read ↗

Pathogenic mechanisms of myotonic dystrophy.Semantic proximity Read ↗

A fly model for the CCUG-repeat expansion of myotonic dystrophy type 2 reveals a novel interaction with MBNL1.Semantic proximity Read ↗

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

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Regulation of Toxic RNA Foci and Mutant <i>DMPK</i> Transcripts: Role of MBNL Proteins and RNA Decay PathwaysSemantic proximity Read ↗

Overexpression of CUG triplet repeat-binding protein, CUGBP1, in mice inhibits myogenesis.Semantic proximity Read ↗

rbFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differencesSemantic proximity Read ↗

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