...Dominique O. Riddell; John C. W. Hildyard; Rachel C. M. Harron; Natasha L. Hornby; Dominic J. Wells; Richard J. Piercy ABSTRACT Duchenne muscular dystrophy (DMD) is a fatal muscle-wasting disease, caused by mutations in the dystrophin gene, characterised by cycles of muscle degeneration...

...Chady H. Hakim; Hsiao T. Yang; Matthew J. Burke; James Teixeira; Gregory J. Jenkins; N. Nora Yang; Gang Yao; Dongsheng Duan ABSTRACT Aged dystrophin-null canines are excellent models for studying experimental therapies for Duchenne muscular dystrophy, a lethal muscle disease caused by dystrophin...

...Naomi Teramoto; Hidetoshi Sugihara; Keitaro Yamanouchi; Katsuyuki Nakamura; Koichi Kimura; Tomoko Okano; Takanori Shiga; Taku Shirakawa; Masafumi Matsuo; Tetsuya Nagata; Masao Daimon; Takashi Matsuwaki; Masugi Nishihara ABSTRACT Dystrophin, encoded by the DMD gene on the X chromosome, stabilizes...

...% bovine serum albumin and 10% fetal bovine serum (F4/80) or 0.1% Triton X-100 and 10% fetal bovine serum (Ly6). Following blocking, sections were incubated with a combination of anti-dystrophin at a dilution of 1:100 and either anti-F4/80 conjugated to Alexa-Fluor-488 (ab6640; Abcam, 1:100) or anti-Ly6...

..., the Dp71 dystrophin. To determine for the first time whether Dp71 loss could affect cerebellar physiology and functions, we have used patch-clamp electrophysiological recordings in acute cerebellar slices and a cerebellum-dependent behavioral test battery addressing cerebellum-dependent motor and non-motor...

...Tingting Sui; Yeh Siang Lau; Di Liu; Tingjun Liu; Li Xu; Yandi Gao; Liangxue Lai; Zhanjun Li; Renzhi Han ABSTRACT Duchenne muscular dystrophy (DMD) is an X-linked muscle-wasting disorder caused by mutations in the dystrophin gene, with an incidence of 1 in 3500 in new male births. Mdx mice...

...Joe W. McGreevy; Chady H. Hakim; Mark A. McIntosh; Dongsheng Duan Duchenne muscular dystrophy (DMD) is a progressive muscle-wasting disorder. It is caused by loss-of-function mutations in the dystrophin gene. Currently, there is no cure. A highly promising therapeutic strategy is to replace...

.... , Hogrel   J. Y. ( 2009 ). Gait analysis using accelerometry in dystrophin-deficient dogs . Neuromuscul. Disord.   19 , 788 – 796 . Barthélémy   I. , Barrey   E. , Aguilar   P. , Uriarte   A. , Le Chevoir   M. , Thibaud   J. L. , Voit   T. , Blot   S. , Hogrel   J. Y...

.... , Weir   A. , Newey   S. E. , Davies   K. E. ( 2002 ). Function and genetics of dystrophin and dystrophin-related proteins in muscle . Physiol. Rev.   82 , 291 – 329 . Brenman   J. E. , Chao   D. S. , Xia   H. , Aldape   K. , Bredt   D. S. ( 1995 ). Nitric oxide...