نتایج جستجو برای: dmd

تعداد نتایج: 4377  

Journal: :Neuromuscular Disorders 2020

Journal: :Lab Animal 2019

2012
Alissa Cyrus Natalie Street Sharon Quary Julie Kable Aileen Kenneson Paul Fernhoff

Purpose. The purpose of this study was to assess the desirability of Duchenne muscular dystrophy (DMD) screening, the effectiveness of the consent process, and the feasibility of conducting DMD screening in a pediatric office. Methods. Infant males who attended a 12-month routine well-child visit at a participating pediatric clinic were screened for DMD. Parents and providers completed post-scr...

Journal: :The Biochemical journal 1982
M J Dunn A H Burghes V Dubowitz

The Ca2+-stimulated Mg2-dependent ATPase activities (Ca2+-ATPase) of erythrocyte-ghost membranes from patients with Duchenne muscular dystrophy (DMD) and carriers of DMD were compared with activities of normal controls. The Ca2+-ATPase activity of DMD-patient ghost preparations was found to follow the same pattern of activation by Ca2+ as the control membranes. However, the Ca2+-ATPase activity...

Journal: :Human molecular genetics 2013
Nikolai Klymiuk Andreas Blutke Alexander Graf Sabine Krause Katinka Burkhardt Annegret Wuensch Stefan Krebs Barbara Kessler Valeri Zakhartchenko Mayuko Kurome Elisabeth Kemter Hiroshi Nagashima Benedikt Schoser Nadja Herbach Helmut Blum Rüdiger Wanke Annemieke Aartsma-Rus Christian Thirion Hanns Lochmüller Maggie C Walter Eckhard Wolf

Duchenne muscular dystrophy (DMD) is caused by mutations in the X-linked dystrophin (DMD) gene. The absence of dystrophin protein leads to progressive muscle weakness and wasting, disability and death. To establish a tailored large animal model of DMD, we deleted DMD exon 52 in male pig cells by gene targeting and generated offspring by nuclear transfer. DMD pigs exhibit absence of dystrophin i...

2017
Tao Li Zhao-jing Zhang Xin Ma Xue Lv Hai Xiao Qian-nan Guo Hong-yan Liu Hong-dan Wang Dong Wu Gui-yu Lou Xin Wang Chao-yang Zhang Shi-xiu Liao

BACKGROUND Patients with Duchenne muscular dystrophy (DMD) usually have severe and fatal symptoms. At present, there is no effective treatment for DMD, thus it is very important to avoid the birth of children with DMD by effective prenatal diagnosis. We identified a de novo DMD gene mutation in a Chinese family, and make a prenatal diagnosis. METHODS First, multiplex ligation-dependent probe ...

2015
Bo Lin Yang Li Lu Han Aaron D. Kaplan Ying Ao Spandan Kalra Glenna C. L. Bett Randall L. Rasmusson Chris Denning Lei Yang

Duchenne muscular dystrophy (DMD) is caused by mutations in the dystrophin gene (DMD), and is characterized by progressive weakness in skeletal and cardiac muscles. Currently, dilated cardiomyopathy due to cardiac muscle loss is one of the major causes of lethality in late-stage DMD patients. To study the molecular mechanisms underlying dilated cardiomyopathy in DMD heart, we generated cardiomy...

2014
Katsuyuki Nakamura Wataru Fujii Masaya Tsuboi Jun Tanihata Naomi Teramoto Shiho Takeuchi Kunihiko Naito Keitaro Yamanouchi Masugi Nishihara

Duchenne muscular dystrophy (DMD) is an X-linked lethal muscle disorder caused by mutations in the Dmd gene encoding Dystrophin. DMD model animals, such as mdx mice and canine X-linked muscular dystrophy dogs, have been widely utilized in the development of a treatment for DMD. Here, we demonstrate the generation of Dmd-mutated rats using a clustered interspaced short palindromic repeats (CRISP...

Journal: :Journal of cell science 1985
J A Witkowski V Dubowitz

Duchenne muscular dystrophy (DMD) is a severe degenerative disorder of skeletal muscle. It has been suggested that an abnormality of the plasma membrane may be responsible for the pathogenesis of DMD, and a number of cell surface changes have been described in DMD muscle fibres and other cell types. Alterations in cell-to-cell and cell-to-substratum adhesiveness have been reported for DMD cells...

2015
Bo Lin Yang Li Lu Han Aaron D. Kaplan Ying Ao Spandan Kalra Glenna C. L. Bett Randall L. Rasmusson Chris Denning

Duchenne muscular dystrophy (DMD) is caused by mutations in the dystrophin gene (DMD), and is characterized by progressive weakness inskeletal andcardiacmuscles.Currently, dilatedcardiomyopathydue to cardiac muscle loss is one of the major causes of lethality in late-stage DMD patients. To study the molecular mechanisms underlying dilated cardiomyopathy in DMD heart, we generated cardiomyocytes...

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