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Start Over Descriptor "CGG-repeat" Journal febs letters
2 results on '"CGG-repeat"'

1. Long CGG-repeat tracts are toxic to human cells: Implications for carriers of Fragile X premutation alleles

Author

Handa, Vaishali, Goldwater, Deena, Stiles, David, Cam, Margaret, Poy, George, Kumari, Daman, and Usdin, Karen

Subjects
*DISEASES, *NEUROLOGIC manifestations of general diseases, *FRAGILE X syndrome, *HEREDITY
Abstract

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 UBE3A. [Copyright &y& Elsevier]

Published
2005
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2. Long CGG-repeat tracts are toxic to human cells: Implications for carriers of Fragile X premutation alleles

Author

Vaishali Handa, Margaret C. Cam, Karen Usdin, George Poy, Deena Goldwater, Daman Kumari, and David Stiles

Subjects
Untranslated region, Biochemistry, Culture Media, Serum-Free, Structural Biology, Fragile X tremor and ataxia syndrome, POF, Annexin A5, Neurotensin, Oligonucleotide Array Sequence Analysis, Genetics, Caspase 8, Premature ovarian failure, Fragile X syndrome, Caspases, Doxycycline, Premutation, Female, medicine.symptom, Paraquat, Heterozygote, congenital, hereditary, and neonatal diseases and abnormalities, Ataxia, Cell Survival, Ubiquitin-Protein Ligases, Blotting, Western, Biophysics, Biology, Cell Line, medicine, UBE3A, Humans, Allele, Molecular Biology, Gene, Alleles, Adaptor Proteins, Signal Transducing, Dose-Response Relationship, Drug, Herbicides, Gene Expression Profiling, CGG-repeat, Cell Biology, Staurosporine, medicine.disease, FMR1, nervous system diseases, Gene Expression Regulation, Fragile X Syndrome, Mutation, 5' Untranslated Regions, Trinucleotide Repeat Expansion
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 UBE3A.

Published
2005

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