نتایج جستجو برای: gaa trinucleotide repeat

تعداد نتایج: 75075  

Journal: :Nature Genetics 2021

Stephen T. Warren was a key contributor to the 1991 discovery of an unstable trinucleotide repeat that expands in families and causes loss function fragile X syndrome.

A Mohseni Meybodi H Gourabi M Chehrazi M Sabbaghian, MA Sadighi Gilani, V Firouzi,

Background Androgen receptor (AR) mediates androgen actions such as initiation and promotion of spermatogenesis and growth of accessory sex organs. There are two trinucleotide polymorphisms (CAG and GGN repeats) in exon1 of AR gene that are vary in length in population. The CAG and GGN repeats association with infertility is still unknown and this study is planned to assess the distribution of ...

2014
Sara Anjomani Virmouni Chiranjeevi Sandi Sahar Al-Mahdawi Mark A. Pook

BACKGROUND Friedreich ataxia (FRDA) is an autosomal recessive neurodegenerative disorder, caused by a GAA repeat expansion mutation within intron 1 of the FXN gene. We have previously established and performed preliminary characterisation of several human FXN yeast artificial chromosome (YAC) transgenic FRDA mouse models containing GAA repeat expansions, Y47R (9 GAA repeats), YG8R (90 and 190 G...

2016
Helen Bergquist Cristina S. J. Rocha Rubén Álvarez-Asencio Chi-Hung Nguyen Mark. W. Rutland C. I. Edvard Smith Liam Good Peter E. Nielsen Rula Zain

Expansion of (GAA)n repeats in the first intron of the Frataxin gene is associated with reduced mRNA and protein levels and the development of Friedreich's ataxia. (GAA)n expansions form non-canonical structures, including intramolecular triplex (H-DNA), and R-loops and are associated with epigenetic modifications. With the aim of interfering with higher order H-DNA (like) DNA structures within...

2017
Susan E Tsutakawa Mark J Thompson Andrew S Arvai Alexander J Neil Steven J Shaw Sana I Algasaier Jane C Kim L David Finger Emma Jardine Victoria J B Gotham Altaf H Sarker Mai Z Her Fahad Rashid Samir M Hamdan Sergei M Mirkin Jane A Grasby John A Tainer

DNA replication and repair enzyme Flap Endonuclease 1 (FEN1) is vital for genome integrity, and FEN1 mutations arise in multiple cancers. FEN1 precisely cleaves single-stranded (ss) 5'-flaps one nucleotide into duplex (ds) DNA. Yet, how FEN1 selects for but does not incise the ss 5'-flap was enigmatic. Here we combine crystallographic, biochemical and genetic analyses to show that two dsDNA bin...

1999
Martin B Delatycki Robert Williamson Susan M Forrest

Friedreich ataxia, an autosomal recessive neurodegenerative disease, is the most common of the inherited ataxias. The recent discovery of the gene that is mutated in this condition, FRDA, has led to rapid advances in the understanding of the pathogenesis of Friedreich ataxia. About 98% of mutant alleles have an expansion of a GAA trinucleotide repeat in intron 1 of the gene. This leads to reduc...

2017
Helen Bergquist Cristina S J Rocha Rubén Álvarez-Asencio Chi-Hung Nguyen Mark W Rutland C I Edvard Smith Liam Good Peter E Nielsen Rula Zain

Expansion of (GAA)n repeats in the first intron of the Frataxin gene is associated with reduced mRNA and protein levels and the development of Friedreich’s ataxia. (GAA)n expansions form non-canonical structures, including intramolecular triplex (H-DNA), and Rloops and are associated with epigenetic modifications. With the aim of interfering with higher order H-DNA (like) DNA structures within ...

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.

2017
William Sproviero Aleksey Shatunov Daniel Stahl Maryam Shoai Wouter van Rheenen Ashley R. Jones Safa Al-Sarraj Peter M. Andersen Nancy M. Bonini Francesca L. Conforti Philip Van Damme Hussein Daoud Maria Del Mar Amador Isabella Fogh Monica Forzan Ben Gaastra Cinzia Gellera Aaron D. Gitler John Hardy Pietro Fratta Vincenzo La Bella Isabelle Le Ber Tim Van Langenhove Serena Lattante Yi-Chung Lee Andrea Malaspina Vincent Meininger Stéphanie Millecamps Richard Orrell Rosa Rademakers Wim Robberecht Guy Rouleau Owen A. Ross Francois Salachas Katie Sidle Bradley N. Smith Bing-Wen Soong Gianni Sorarù Giovanni Stevanin Edor Kabashi Claire Troakes Christine van Broeckhoven Jan H. Veldink Leonard H. van den Berg Christopher E. Shaw John F. Powell Ammar Al-Chalabi

We investigated a CAG trinucleotide repeat expansion in the ATXN2 gene in amyotrophic lateral sclerosis (ALS). Two new case-control studies, a British dataset of 1474 ALS cases and 567 controls, and a Dutch dataset of 1328 ALS cases and 691 controls were analyzed. In addition, to increase power, we systematically searched PubMed for case-control studies published after 1 August 2010 that invest...

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