Literature DB >> 11252709

Fragile X full mutations are more similar in siblings than in unrelated patients: further evidence for a familial factor in CGG repeat dynamics.

R W Burman1, K S Anoe, B W Popovich.   

Abstract

PURPOSE: We sought to compare patterns of full mutation repeat-length variability in the peripheral blood DNA of patients with fragile X syndrome to determine whether siblings possess mutation patterns more similar than those of unrelated patients.
METHODS: Mutation patterns were visualized by Southern blot analysis and captured digitally with a phosphor imager. Novel comparison strategies based on overlapping profile plots and calculation of weighted mean CGG repeat values were used to assess mutation pattern similarity.
RESULTS: Within the population that we analyzed of 56 patients with full mutation, mutation patterns were found to be more similar in siblings than in unrelated patients.
CONCLUSION: These results indicate that repeat-length variability may be generated in a nonrandom manner and that familial factors influence this process.

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Mesh:

Year:  2000        PMID: 11252709     DOI: 10.1097/00125817-200007000-00007

Source DB:  PubMed          Journal:  Genet Med        ISSN: 1098-3600            Impact factor:   8.822


  2 in total

1.  Paternally transmitted FMR1 alleles are less stable than maternally transmitted alleles in the common and intermediate size range.

Authors:  Amy K Sullivan; Dana C Crawford; Elizabeth H Scott; Mary L Leslie; Stephanie L Sherman
Journal:  Am J Hum Genet       Date:  2002-05-03       Impact factor: 11.025

Review 2.  FMR1 and the fragile X syndrome: human genome epidemiology review.

Authors:  D C Crawford; J M Acuña; S L Sherman
Journal:  Genet Med       Date:  2001 Sep-Oct       Impact factor: 8.822

  2 in total

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