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Article

A MutSβ-Dependent Contribution of MutSα to Repeat Expansions in Fragile X Premutation Mice?

PLoS genetics - 1 Jul 2016

Zhao Xiao-Nan, Lokanga Rachel, Allette Kimaada, Gazy Inbal, Wu Di, Usdin Karen

Abstract excerpt

The fragile X-related disorders result from expansion of a CGG/CCG microsatellite in the 5' UTR of the FMR1 gene. We have previously demonstrated that the MSH2/MSH3 complex, MutSβ, that is important for mismatch repair, is essential for almost all expansions in a mouse model of these disorders. Here we show that the MSH2/MSH6 complex, MutSα also contributes to the production of both germ line and somatic...

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Topics

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Share to Fragile X Mental Retardation Protein
Share to Fragile X Syndrome

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Mice, Transgenic

Share to MutS DNA Mismatch-Binding Protein

Share to MutS Homolog 2 Protein

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Source

Open in PubMed Open full text

Identifiers

PMID: 27427765
DOI: 10.1371/journal.pgen.1006190
PMCID: PMC4948851

Genes

MSH2
MSH3
MSH6
FMR1

Provenance

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

Related research

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