نتایج جستجو برای: friedreich ataxia

تعداد نتایج: 17893  

Journal: :iranian journal of basic medical sciences 0
mohammad mehdi heidari department of biology, science school, yazd university, yazd, iran. mehri khatami department of biology, science school, yazd university, yazd, iran.

objective(s) the mitochondrial defects in friedreich's ataxia have been reported in many researches. mitochondrial dna is one of the candidates for defects in mitochondrion, and complex i is the first and one of the largest catalytic complexes of oxidative phosphorylation (oxphos) system. materials and methods we searched the mitochondrial nd4l gene for mutations by ttge and sequencing on ...

Journal: :FASEB journal : official publication of the Federation of American Societies for Experimental Biology 2008
Robert D Wells

Friedreich ataxia, the most common inherited ataxia, is caused by the transcriptional silencing of the FXN gene, which codes for the 210 amino acid frataxin, a mitochondrial protein involved in iron-sulfur cluster biosynthesis. The expansion of the GAA x TTC tract in intron 1 to as many as 1700 repeats elicits the transcriptional silencing by the formation of non-B DNA structures (triplexes or ...

Journal: :Brain : a journal of neurology 2008
Michael C Fahey Phillip D Cremer Swee T Aw Lynette Millist Michael J Todd Owen B White Michael Halmagyi Louise A Corben Veronica Collins Andrew J Churchyard Kim Tan Lionel Kowal Martin B Delatycki

Friedreich ataxia (FRDA), the commonest of the inherited ataxias, is a multisystem neurodegenerative condition that affects ocular motor function. We assessed eye movement abnormalities in 20 individuals with genetically confirmed FRDA and compared these results to clinical measures. All subjects were assessed with infrared oculography. Fifteen individuals underwent a full protocol of eye movem...

Journal: :AJNR. American journal of neuroradiology 2015
T A Santos C E B Maistro C B Silva M S Oliveira M C França G Castellano

BACKGROUND AND PURPOSE Texture analysis is an image processing technique that can be used to extract parameters able to describe meaningful features of an image or ROI. Texture analysis based on the gray level co-occurrence matrix gives a second-order statistical description of the image or ROI. In this work, the co-occurrence matrix texture approach was used to extract information from brain M...

Journal: :Blood 2007
Nathalie Boddaert Kim Hanh Le Quan Sang Agnès Rötig Anne Leroy-Willig Serge Gallet Francis Brunelle Daniel Sidi Jean-Christophe Thalabard Arnold Munnich Z Ioav Cabantchik

Genetic disorders of iron metabolism and chronic inflammation often evoke local iron accumulation. In Friedreich ataxia, decreased iron-sulphur cluster and heme formation leads to mitochondrial iron accumulation and ensuing oxidative damage that primarily affects sensory neurons, the myocardium, and endocrine glands. We assessed the possibility of reducing brain iron accumulation in Friedreich ...

2010
Sylvia Boesch Brigitte Sturm Wolfgang Nachbauer Sascha Hering Hannes Steinkellner Rainer Schneider Werner Poewe Barbara Scheiber-Mojdehkar

In vitro and in vivo studies have provided evidence for neuroprotective properties of Erythropoietin in neurodegenerative disorders. Although the magnitude of effect is still controversial, very recent findings point to neuronal protection in the central nervous system by Erythropoietins. Erythropoietin is a powerful growth factor which enhances cellular size and ultimatively increases the numb...

Journal: :The American Journal of Human Genetics 1999

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