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Article

Muscleblind protein, MBNL1/EXP, binds specifically to CHHG repeats

6 Jan 2004

Abstract excerpt

Myotonic dystrophy (DM) type 1 is caused by an expansion of a CTG repeat in the DMPK gene and type 2 by a CCTG repeat in the ZNF9 gene. Previous reports have suggested that transcripts containing expanded CUG/CCUG repeats might have toxic gain-of-function effects, probably affecting the function of RNA-binding proteins in the pathogenesis of DM. Here, it was attempted to compare the RNA-binding properties of...

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Identifiers

PMID: 14722159
DOI: 10.1093/hmg/ddh056

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Click a neighbor to travelStep 1 · 12 closest

Muscleblind protein, MBNL1/EXP, binds specifically to CHHG repeatsPMID 14722159Read ↗

The four Zn fingers of MBNL1 provide a flexible platform for recognition of its RNA binding elementsSemantic proximity Read ↗

MBNL1–RNA Recognition: Contributions of MBNL1 Sequence and RNA ConformationSemantic proximity Read ↗

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

Pathogenic mechanisms of myotonic dystrophy.Semantic proximity Read ↗

Muscleblind-like 1 interacts with RNA hairpins in splicing target and pathogenic RNAsSemantic proximity Read ↗

MBNL binds similar RNA structures in the CUG repeats of myotonic dystrophy and its pre-mRNA substrate cardiac troponin TSemantic proximity Read ↗

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.Semantic proximity Read ↗

MBNL1 binds GC motifs embedded in pyrimidines to regulate alternative splicing.Semantic proximity Read ↗

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

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

Alternative splicing regulation by Muscleblind proteins: from development to diseaseSemantic proximity Read ↗

(CTG)n repeat-mediated dysregulation of MBNL1 and MBNL2 expression during myogenesis in DM1 occurs already at the myoblast stageSemantic proximity Read ↗

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