نتایج جستجو برای: gaa trinucleotide repeat

تعداد نتایج: 75075  

Journal: :Diabetes 2000
J Hebinck C Hardt L Schöls M Vorgerd L Briedigkeit C R Kahn M Ristow

Friedreich's ataxia (FA) is an autosomal recessive disease that has been attributed to a GAA triplet repeat expansion in the first intron of the X25/frataxin gene. Impaired glucose tolerance is present in up to 39% of FA patients, and clinically apparent diabetes is seen in approximately 18% of the affected individuals. Subjects carrying the X25/frataxin GAA repeat in a heterozygous state do no...

2017
Ashlee Long Jill S Napierala Urszula Polak Lauren Hauser Arnulf H Koeppen David R Lynch Marek Napierala

Friedreich's ataxia (FRDA) is a genetic neurodegenerative disorder caused by transcriptional silencing of the frataxin gene (FXN) due to expansions of GAA repeats in intron 1. FRDA manifests with multiple symptoms, which may include ataxia, cardiomyopathy and diabetes mellitus. Expanded GAA tracts are genetically unstable, exhibiting both expansions and contractions. GAA length correlates with ...

2014
Beatriz A. Santillan Christopher Moye David Mittelman John H. Wilson

Trinucleotide repeats can be highly unstable, mutating far more frequently than point mutations. Repeats typically mutate by addition or loss of units of the repeat. CAG repeat expansions in humans trigger neurological diseases that include myotonic dystrophy, Huntington disease, and several spinocerebellar ataxias. In human cells, diverse mechanisms promote CAG repeat instability, and in mice,...

Journal: :Archives of neurology 2012
Mathieu Anheim Louise-Laure Mariani Patrick Calvas Emmanuel Cheuret Fabien Zagnoli Sylvie Odent Claire Seguela Cecilia Marelli Marlène Fritsch Jean-Pierre Delaunoy Alexis Brice Alexandra Dürr Michel Koenig

BACKGROUND Friedreich ataxia (FA) is the most frequent type of autosomal recessive cerebellar ataxia, occurring at a mean age of 16 years. Nearly 98% of patients with FA present with homozygous GAA expansions in the FXN gene. The remaining patients are compound heterozygous for an expansion and a point mutation. Patients who are compound heterozygous for an exonic deletion and an expansion are ...

نمودار تعداد نتایج جستجو در هر سال

با کلیک روی نمودار نتایج را به سال انتشار فیلتر کنید