Skip to main content
U.S. Department of Energy
Office of Scientific and Technical Information

Trinucleotide repeats at the FRAXF locus: Frequency and distribution in the general population

Journal Article · · American Journal of Medical Genetics
;  [1]
  1. Ongwanada Resource Centre, Ontario (Canada); and others

FRAXF the third X-chromosomal fragile site to be cloned, has been shown to harbor a polymorphic compound triplet array: (GC-CGTC){sub n} (GCC){sub n}. Expansion and methylation of the GCC-repeat and the neighboring CpG-rich region result in chromosomal fragility. DNAs from 500 anonymous consecutive newborn males were examined to determine the incidence of various repeat numbers. The range of repeats was from 10-38, with the most common alleles having 14 (52.7%), 12 (16.6%), 21 (9.0%), and 22 (5.2%) triplets. Based on the distribution of repeat numbers, we suggest that the 21-repeat allele resulted from hairpin formation involving 7 GCC-repeats in a 14-repeat allele, accompanied by polymerase slippage. Examination of dinucleotide repeats near the FRAXF repeat will be important in testing this hypothesis. Since the clinical phenotype, if any, of FRAXF is unknown, this database will also be valuable for comparisons with repeat numbers in individuals from special populations. 20 refs., 1 fig., 1 tab.

OSTI ID:
478902
Journal Information:
American Journal of Medical Genetics, Journal Name: American Journal of Medical Genetics Journal Issue: 2 Vol. 64; ISSN 0148-7299; ISSN AJMGDA
Country of Publication:
United States
Language:
English

Similar Records

Trinucleotide repeat expansion in the FRAXE locus is not common among institutionalized individuals with non-specific developmental disabilities
Journal Article · Fri Aug 09 00:00:00 EDT 1996 · American Journal of Medical Genetics · OSTI ID:478901

A survey of FRAXE allele sizes in three populations
Journal Article · Fri Aug 09 00:00:00 EDT 1996 · American Journal of Medical Genetics · OSTI ID:478900

Hairpins are formed by the single DNA strands of the fragile X triplet repeats: Structure and biological implications
Journal Article · Tue May 23 00:00:00 EDT 1995 · Proceedings of the National Academy of Sciences of the United States of America · OSTI ID:75386