نتایج جستجو برای: utrophin

تعداد نتایج: 527  

Journal: :Neuroreport 2007
Ramses Ilarraza-Lomeli Bulmaro Cisneros-Vega Maria de Lourdes Cervantes-Gomez Dominique Mornet Cecilia Montañez

Function of dystrophin Dp71 isoforms is unknown but seems related to neurite outgrowth and synapse formation. To evaluate Dp71 role in myoneural synapses, we established a coculture model using PC12 cells and L6 myotubes and analyzed expression and localization of Dp71 and related proteins, utrophin and beta-dystroglycan, in PC12 cells. Confocal microscopy showed Dp71d isoform in PC12 nuclei, g...

Journal: :Cell 1997
Anne E Deconinck Jill A Rafael Judith A Skinner Susan C Brown Allyson C Potter Laurent Metzinger Diana J Watt J.George Dickson Jonathon M Tinsley Kay E Davies

The absence of dystrophin at the muscle membrane leads to Duchenne muscular dystrophy (DMD), a severe muscle-wasting disease that is inevitably fatal in early adulthood. In contrast, dystrophin-deficient mdx mice appear physically normal despite their underlying muscle pathology. We describe mice deficient for both dystrophin and the dystrophin-related protein utrophin. These mice show many sig...

Journal: :Biochemical and biophysical research communications 2006
Rahul C Bhosle Daniel E Michele Kevin P Campbell Zhenlin Li Richard M Robson

Synemin is a unique, very large intermediate filament (IF) protein present in all types of muscle cells, which forms heteropolymeric intermediate filaments (IFs) with the major IF proteins desmin and/or vimentin. We show herein that tissue-purified avian synemin directly interacts with both dystrophin and utrophin, and that specific expressed regions of both of the mammalian (human) synemin iso...

2012
Mike J.F. Broderick Andrey Bobkov Steve J. Winder

Structural analyses of actin binding regions comprising tandem calponin homology domains alone and when bound to F-actin have revealed a number of different conformations with calponin homology domains in 'open' and 'closed' positions. In an attempt to resolve these issues we have examined the properties of the utrophin actin binding domain in open and closed conformations in order to verify th...

Journal: :Neuromuscular Disorders 2012
Maaike van Putten Darshan Kumar Margriet Hulsker Willem M.H. Hoogaars Jaap J. Plomp Annemarieke van Opstal Maarten van Iterson Peter Admiraal Gert-Jan B. van Ommen Peter A.C. ‘t Hoen Annemieke Aartsma-Rus

The genetic defect of mdx mice resembles that of Duchenne muscular dystrophy, although their functional performance and life expectancy is nearly normal. By contrast, mice lacking utrophin and dystrophin (mdx/utrn -/-) are severely affected and die prematurely. Mice with one utrophin allele (mdx/utrn +/-) are more severely affected than mdx mice, but outlive mdx/utrn -/- mice. We subjected mdx/...

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