نتایج جستجو برای: trinucleotide expansion

تعداد نتایج: 142243  

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2000
C Jankowski F Nasar D K Nag

Expansion of trinucleotide repeats is associated with a growing number of human diseases. The mechanism and timing of expansion of the repeat tract are poorly understood. In humans, trinucleotide repeats show extreme meiotic instability, and expansion of the repeat tract has been suggested to occur in the germ-line mitotic divisions or postmeiotically during early divisions of the embryo. Studi...

Journal: :Brain : a journal of neurology 2004
Caroline J Moore Eileen M Daly Flora Tassone Carolyn Tysoe Nicole Schmitz Virginia Ng Xavier Chitnis Philip McGuire John Suckling Kay E Davies Randi J Hagerman Paul J Hagerman Kieran C Murphy Declan G M Murphy

Expanded trinucleotide repeats are associated with several neuropsychiatric disorders, including fragile X syndrome (FraX) which is the most common inherited form of mental retardation. It is currently thought that FraX results from having >200 CGG trinucleotide repeats, with consequent methylation of the fragile X mental retardation gene (FMR1) and loss of FMR1 protein (FMRP). Pre-mutation car...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2003
Song-Ro Yoon Louis Dubeau Margot de Young Nancy S Wexler Norman Arnheim

Single-molecule DNA analysis of testicular germ cells isolated by laser capture microdissection from two Huntington disease patients showed that trinucleotide repeat expansion mutations were present before the end of the first meiotic division, and some mutations were present even before meiosis began. Most of the larger Huntington disease mutations were found in the postmeiotic cell population...

Journal: :PLoS Computational Biology 2007
Shai Kaplan Shalev Itzkovitz Ehud Y. Shapiro

Trinucleotide hereditary diseases such as Huntington disease and Friedreich ataxia are cureless diseases associated with inheriting an abnormally large number of DNA trinucleotide repeats in a gene. The genes associated with different diseases are unrelated and harbor a trinucleotide repeat in different functional regions; therefore, it is striking that many of these diseases have similar corre...

2012
Karen Kelley Shin-Ju E. Chang Shi-Lung Lin

The majority of the human genome is comprised of non-coding DNA, which frequently contains redundant microsatellite-like trinucleotide repeats. Many of these trinucleotide repeats are involved in triplet repeat expansion diseases (TREDs) such as fragile X syndrome (FXS). After transcription, the trinucleotide repeats can fold into RNA hairpins and are further processed by Dicer endoribonuclases...

Journal: :Clinical chemistry 1996
M Muglia O Leone G Annesi A L Gabriele E Imbrogno C Grandinetti F L Conforti F Naso C Brancati

Huntington disease (HD) is a neurodegenerative disorder caused by an expanded trinucleotide repeat (CAG)n located at the 5' end of the novel IT15 gene. Discovery of this expansion allows the molecular diagnosis of HD by measuring repeat length. We applied a simple nonisotopic method to detect (CAG)n repeats, avoiding both radioactive and Southern transfer analysis. The assay is based on direct ...

Journal: :Journal of medical genetics 1995
L Martorell J M Martinez N Carey K Johnson M Baiget

Myotonic dystrophy (DM) is associated with an underlying CTG trinucleotide repeat expansion at a locus on chromosome 19q13.3. We have determined the repeat length in 23 DM patients with varying clinical severity of symptoms and various sizes of repeat amplification. We confirm that as in previous studies there is no strong correlation between repeat length and clinical symptoms but find that th...

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