نتایج جستجو برای: trinucleotide expansion

تعداد نتایج: 142243  

Journal: :Proceedings of the National Academy of Sciences of the United States of America 1997
F R Goodman S Mundlos Y Muragaki D Donnai M L Giovannucci-Uzielli E Lapi F Majewski J McGaughran C McKeown W Reardon J Upton R M Winter B R Olsen P J Scambler

Synpolydactyly (SPD) is a dominantly inherited congenital limb malformation. Typical cases have 3/4 finger and 4/5 toe syndactyly, with a duplicated digit in the syndactylous web, but incomplete penetrance and variable expressivity are common. The condition has recently been shown to be caused by expansions of an imperfect trinucleotide repeat sequence encoding a 15-residue polyalanine tract in...

2016
Piera Cilli Ilenia Ventura Anna Minoprio Ettore Meccia Alberto Martire Samuel H. Wilson Margherita Bignami Filomena Mazzei

DNA trinucleotide repeat (TNR) expansion underlies several neurodegenerative disorders including Huntington's disease (HD). Accumulation of oxidized DNA bases and their inefficient processing by base excision repair (BER) are among the factors suggested to contribute to TNR expansion. In this study, we have examined whether oxidation of the purine dNTPs in the dNTP pool provides a source of DNA...

2015
Marta Olejniczak Martyna O Urbanek Wlodzimierz J Krzyzosiak

Trinucleotide repeat expansion disorders (TREDs) are a group of dominantly inherited neurological diseases caused by the expansion of unstable repeats in specific regions of the associated genes. Expansion of CAG repeat tracts in translated regions of the respective genes results in polyglutamine- (polyQ-) rich proteins that form intracellular aggregates that affect numerous cellular activities...

Journal: :Nucleic acids research 1996
J Ji N J Clegg K R Peterson A L Jackson C D Laird L A Loeb

The human fragile-X syndrome, a major cause of inherited mental retardation, is associated with expansion of the trinucleotide repeat GGC:GCC. Repetitive sequences in DNA are subject to slippage during catalysis by DNA polymerases. We characterized the extent of slippage of synthetic GGC:GCC repeats by various DNA polymerases: Taq DNA polymerase, Klenow fragment of DNA polymerase I, DNA Sequenc...

2015
Jill M. Beaver Yanhao Lai Meng Xu Astrid H. Casin Eduardo E. Laverde Yuan Liu

Base excision repair (BER) of an oxidized base within a trinucleotide repeat (TNR) tract can lead to TNR expansions that are associated with over 40 human neurodegenerative diseases. This occurs as a result of DNA secondary structures such as hairpins formed during repair. We have previously shown that BER in a TNR hairpin loop can lead to removal of the hairpin, attenuating or preventing TNR e...

Journal: :Journal of the neurological sciences 1995
G J Jöbsis E S Louwerse M de Visser R A Wolterman P A Bolhuis H F Busch H T Brüggenwirth F Baas W M Wiersinga J H Koelman

Kennedy disease is caused by an enlarged trinucleotide repeat sequence within the androgen receptor gene. We report here seven male patients with a benign motor neuron syndrome highly analogous to Kennedy disease but with a normal trinucleotide repeat.

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