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Article

ATM and ATR protect the genome against two different types of tandem repeat instability in Fragile X premutation mice.

Nucleic acids research - 1 Oct 2009

Entezam Ali, Usdin Karen

Abstract excerpt

Expansion of a tandem repeat tract is responsible for the Repeat Expansion diseases, a group of more than 20 human genetic disorders that includes those like Fragile X (FX) syndrome that result from repeat expansion in the FMR1 gene. We have previously shown that the ATM and Rad3-related (ATR) ch...

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Source

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Identifiers

PMID: 19710035
DOI: 10.1093/nar/gkp666
PMCID: PMC2770655

Genes

ATM
ATR
FMR1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026. Prepared record publication-19710035-2026-09-02.2. Manifest publication-graph-cohort-2026-09-02.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 2 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

ATM and ATR protect the genome against two different types of tandem repeat instability in Fragile X premutation mice.PMID 19710035Read ↗

ATR protects the genome against CGG.CCG-repeat expansion in Fragile X premutation mice.Semantic proximity Read ↗

Heterozygosity for a hypomorphic Polβ mutation reduces the expansion frequency in a mouse model of the Fragile X-related disorders.Semantic proximity Read ↗

Somatic expansion in mouse and human carriers of fragile X premutation alleles.Semantic proximity Read ↗

The transcription-coupled repair protein ERCC6/CSB also protects against repeat expansion in a mouse model of the fragile X premutation.Semantic proximity Read ↗

The mismatch repair protein MSH2 is rate limiting for repeat expansion in a fragile X premutation mouse model.Semantic proximity Read ↗

Expansion of the CGG repeat in fragile X in the FMR1 gene depends on the sex of the offspring.Semantic proximity Read ↗

FMR1 premutation allele (CGG)81 is stable in mice.Semantic proximity Read ↗

A hypomorphic mutation reveals a stringent requirement for the ATM checkpoint protein in telomere protection during early cell division in Drosophila.Semantic proximity Read ↗

Repeat expansion in a Fragile X model is independent of double strand break repair mediated by Pol θ, Rad52, Rad54l or Rad54bSemantic proximity Read ↗

Gender and cell-type-specific effects of the transcription-coupled repair protein, ERCC6/CSB, on repeat expansion in a mouse model of the fragile X-related disorders.Semantic proximity Read ↗

FMR1 CGG-repeat instability in single sperm and lymphocytes of fragile-X premutation males.Semantic proximity Read ↗

Fully expanded FMR1 CGG repeats exhibit a length- and differentiation-dependent instability in cell hybrids that is independent of DNA methylation.Semantic proximity Read ↗

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