نتایج جستجو برای: facioscapulohumeral muscular dystrophy

تعداد نتایج: 52771  

2003
JOHN P. BALLANTYNE

The concept of the muscular dystrophies as primary degenerative diseases of muscle has been increasingly challenged in recent years (Fenichel et al., 1967; Gardner-Medwin et al., 1967; Munsat et al., 1972; Gallup and Dubowitz, 1973). Considerable support for the neurogenic hypothesis in muscular dystrophy has come from the results obtained using an electrophysiological technique for the estimat...

Journal: :Journal of cell science 2013
Alexandros Xynos Maria Victoria Neguembor Roberta Caccia Danilo Licastro Alessandro Nonis Clelia Di Serio Elia Stupka Davide Gabellini

Overexpression of facioscapulohumeral muscular dystrophy region gene 1 (FRG1) in mice, frogs and worms leads to muscular and vascular abnormalities. Nevertheless, the mechanism that follows FRG1 overexpression and finally leads to muscular defects is currently unknown. Here, we show that the earliest phenotype displayed by mice overexpressing FRG1 is a postnatal muscle-growth defect. Long befor...

Journal: :Journal of neurology, neurosurgery, and psychiatry 1965
D D BARWICK J W OSSELTON J N WALTON

Recent reports by a number of authors have suggested that electroencephalographic (E.E.G.) abnormalities are often found in patients with progressive muscular dystrophy. Winfield, Britt, and Raskind (1958) reported the finding of nine abnormal records in 12 children with muscular dystrophy subjected to E.E.G. study. The abnormalities consisted of the pattern designated by Gibbs and Gibbs (1952)...

Journal: :Human mutation 2009
Jessica C de Greef Richard J L F Lemmers Baziel G M van Engelen Sabrina Sacconi Shannon L Venance Rune R Frants Rabi Tawil Silvère M van der Maarel

Facioscapulohumeral muscular dystrophy (FSHD), caused by partial deletion of the D4Z4 macrosatellite repeat on chromosome 4q, has a complex genetic and epigenetic etiology. To develop FSHD, D4Z4 contraction needs to occur on a specific genetic background. Only contractions associated with the 4qA161 haplotype cause FSHD. In addition, contraction of the D4Z4 repeat in FSHD patients is associated...

Journal: :Human molecular genetics 2015
Richard J L F Lemmers Jelle J Goeman Patrick J van der Vliet Merlijn P van Nieuwenhuizen Judit Balog Marianne Vos-Versteeg Pilar Camano Maria Antonia Ramos Arroyo Ivonne Jerico Mark T Rogers Daniel G Miller Meena Upadhyaya Jan J G M Verschuuren Adolfo Lopez de Munain Arregui Baziel G M van Engelen George W Padberg Sabrina Sacconi Rabi Tawil Stephen J Tapscott Bert Bakker Silvère M van der Maarel

Facioscapulohumeral muscular dystrophy (FSHD: MIM#158900) is a common myopathy with marked but largely unexplained clinical inter- and intra-familial variability. It is caused by contractions of the D4Z4 repeat array on chromosome 4 to 1-10 units (FSHD1), or by mutations in the D4Z4-binding chromatin modifier SMCHD1 (FSHD2). Both situations lead to a partial opening of the D4Z4 chromatin struct...

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