Results 1 to 10 of about 35,595 (240)
Nature Communications, 2019 Dystrophin-deficient mice are used to test corrective strategies for Duchenne muscular dystrophy, but evaluation of dystrophin expression requires collection of tissue samples from specific muscles and time points.
Leonela Amoasii +11 more
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Dystrophin-Deficient Cardiomyopathy.
Journal of the American College of Cardiology, 2016 Dystrophinopathies are a group of distinct neuromuscular diseases that result from mutations in the structural cytoskeletal Dystrophin gene. Dystrophinopathies include Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD), X-linked dilated cardiomyopathy, as well as DMD and BMD female carriers.
F. Kamdar, D. Garry
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Lentiviral vectors can be used for full-length dystrophin gene therapy
Scientific Reports, 2017 Duchenne Muscular Dystrophy (DMD) is caused by a lack of dystrophin expression in patient muscle fibres. Current DMD gene therapy strategies rely on the expression of internally deleted forms of dystrophin, missing important functional domains.
John R. Counsell +10 more
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Function and genetics of dystrophin and dystrophin-related proteins in muscle. [PDF]
Physiological Reviews, 2002 The X-linked muscle-wasting disease Duchenne muscular dystrophy is caused by mutations in the gene encoding dystrophin. There is currently no effective treatment for the disease; however, the complex molecular pathology of this disorder is now being ...
D. Blake +3 more
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Pediatric Neurology Briefs, 1992 Dystrophin, the product of the Duchenne muscular dystrophy gene, was studied in human fetal skeletal muscle from 9 to 26 weeks of gestation at the Jerry Lewis Muscle Research Centre, Hammersmith Hospital, London.
J Gordon Millichap
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N‐terminal domain of dystrophin [PDF]
FEBS Letters, 1994 Contro‐versial experiments have been published on calmodulin binding of dystrophin. In this study, we used recombinant proteins and the techniques of affinity chromatography and ELISA to show that the N‐terminal part of dystrophin binds calmodulin specifically in a calcium‐dependent manner. The calcium‐dependent interaction of calmodulin and dystrophin
Armelle Bonet‐Kerrache +2 more
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The importance of dystrophin and the dystrophin associated proteins in vascular smooth muscle
Frontiers in Physiology, 2022 This review details the role of dystrophin and the dystrophin associated proteins (DAPs) in the vascular smooth muscle. Dystrophin is most comprehensively studied in the skeletal muscle due to serious symptoms found related to the skeletal muscle of ...
Katherine M. Kaplan, Kathleen G. Morgan
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Dystrophinopathy in the paravertebral muscle of adolescent idiopathic scoliosis: a prospective case-control study in China [PDF]
Asian Spine JournalStudy Design A prospective case-control study. Purpose This prospective case-control study aimed to analyze the paravertebral muscle changes in patients with adolescent idiopathic scoliosis (AIS) and determine paravertebral myopathological changes ...
Junyu Li +7 more
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Dystrophin and dystrophin-associated protein in muscles and nerves from monkey
European Journal of Histochemistry, 2009 Since all organs (i.e. skeletal, cardiac, smooth muscles and sciatic nerve) are never only taken from a single patient, all these tissues were obtained from one cynomolgus monkey, a model closely resembling humans.
M Royuela +8 more
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The role of the dystrophin glycoprotein complex in muscle cell mechanotransduction
Communications Biology, 2022 Dystrophin is the central protein of the dystrophin-glycoprotein complex (DGC) in skeletal and heart muscle cells. Dystrophin connects the actin cytoskeleton to the extracellular matrix (ECM).
D. Wilson, A. Tinker, T. Iskratsch
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