Long CGG‐repeat tracts are toxic to human cells: Implications for carriers of Fragile X premutation alleles
- 18 April 2005
- journal article
- research article
- Published by Wiley in FEBS Letters
- Vol. 579 (12) , 2702-2708
- https://doi.org/10.1016/j.febslet.2005.04.004
Abstract
People with 59–200 CGG·CCG-repeats in the 5′ UTR of one of their FMR1 genes are at risk for Fragile X tremor and ataxia syndrome. Females are also at risk for premature ovarian failure. These symptoms are thought to be due to the presence of the repeats at the DNA and/or RNA level. We show here that long transcribed but untranslated CGG-repeat tracts are toxic to human cells and alter the expression of a wide variety of different genes including caspase-8, CYFIP, Neurotensin and UBE3AKeywords
This publication has 31 references indexed in Scilit:
- Expansion of the Fragile X CGG Repeat in Females with Premutation or Intermediate AllelesAmerican Journal of Human Genetics, 2003
- Neuronal intranuclear inclusions in a new cerebellar tremor/ataxia syndrome among fragile X carriersBrain, 2002
- Intention tremor, parkinsonism, and generalized brain atrophy in male carriers of fragile XNeurology, 2001
- Premature ovarian failure in the fragile X syndromeAmerican Journal of Medical Genetics, 2000
- Fragile X premutation is a significant risk factor for premature ovarian failure: The international collaborative POF in fragile X study?preliminary dataAmerican Journal of Medical Genetics, 1999
- Studies of FRAXA and FRAXE in women with premature ovarian failure.Journal of Medical Genetics, 1998
- FRAXA premutation associated with premature ovarian failureAmerican Journal of Medical Genetics, 1996
- Fragile X premutations in familial premature ovarian failureThe Lancet, 1995
- Mapping of DNA Instability at the Fragile X to a Trinucleotide Repeat Sequence P(CCG) nScience, 1991
- Fragile X Genotype Characterized by an Unstable Region of DNAScience, 1991