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Potassium bromate, a potent DNA oxidizing agent, exacerbates germline repeat expansion in a fragile X premutation mouse model
- Source :
- Human Mutation.
- Publication Year :
- 2010
- Publisher :
- Hindawi Limited, 2010.
-
Abstract
- Tandem repeat expansion is responsible for the Repeat Expansion Diseases, a group of human genetic disorders that includes Fragile X syndrome (FXS). FXS results from expansion of a premutation (PM) allele having 55-200 CGG.CCG-repeats in the 5' UTR of the FMR1 gene. The mechanism of expansion is unknown. We have treated FX PM mice with potassium bromate (KBrO(3)), a potent DNA oxidizing agent. We then monitored the germline and somatic expansion frequency in the progeny of these animals. We show here that KBrO(3) increased both the level of 8-oxoG in the oocytes of treated animals and the germline expansion frequency. Our data thus suggest that oxidative damage may be a factor that could affect expansion risk in humans.
- Subjects :
- congenital, hereditary, and neonatal diseases and abnormalities
Flap Endonucleases
DNA repair
DNA damage
Biology
Article
Germline
Mice
chemistry.chemical_compound
Genetics
medicine
Animals
Humans
Allele
Genetics (clinical)
Mice, Knockout
Bromates
Deoxyguanosine
medicine.disease
Molecular biology
FMR1
Fragile X syndrome
Disease Models, Animal
chemistry
8-Hydroxy-2'-Deoxyguanosine
Tandem Repeat Sequences
Fragile X Syndrome
Trinucleotide Repeat Expansion
Trinucleotide repeat expansion
Potassium bromate
DNA Damage
Subjects
Details
- ISSN :
- 10981004 and 10597794
- Database :
- OpenAIRE
- Journal :
- Human Mutation
- Accession number :
- edsair.doi.dedup.....c098ccf604a688694c85bc78fef083f2