Regenerative biomarkers for Duchenne muscular dystrophy
Neural Regeneration Research, 2019 Skeletal muscle has an extraordinary capacity to regenerate after injury and trauma. The muscle repair mechanism is a complex process orchestrated by multiple steps.
Simon Guiraud, Kay E Davies
doaj +1 more source
Characterisation of alpha-dystrobrevin in muscle [PDF]
, 1998 Dystrophin-related and associated proteins are important for the formation and maintenance of the mammalian neuromuscular junction. Initial studies in the electric organ of Torpedo californica showed that the dystrophin-related protein dystrobrevin (87K)
Blake, D. J. +4 more
core
Artificial restoration of the linkage between laminin and dystroglycan ameliorates the disease progression of MDC1A muscular dystrophy at all stages [PDF]
, 2005 Laminin-α2 deficient congenital muscular dystrophy, classified as MDC1A, is a severe progressive muscle-wasting disease that leads to death in early childhood.
Meinen, Sarina
core +1 more source
Neurobiology of Disease, 1999 Duchenne muscular dystrophy (DMD), a severe X-linked recessive disorder which results in progressive muscle degeneration, is due to a lack of dystrophin, a membrane cytoskeletal protein.
Emmanuel Chaubourt +5 more
doaj
An Ankyrin-Based Mechanism for Functional Organization of Dystrophin and Dystroglycan [PDF]
, 2008 Summaryβ-dystroglycan (DG) and the dystrophin-glycoprotein complex (DGC) are localized at costameres and neuromuscular junctions in the sarcolemma of skeletal muscle.
Ayalon, Gai +3 more
core +1 more source
Micro-utrophin Improves Cardiac and Skeletal Muscle Function of Severely Affected D2/mdx Mice
Molecular Therapy: Methods & Clinical Development, 2018 Duchenne muscular dystrophy (DMD) is an X-linked muscle-wasting disease caused by mutations in the dystrophin gene. DMD boys are wheelchair-bound around 12 years and generally survive into their twenties.
Tahnee L. Kennedy +10 more
doaj
Immunostaining of Dystrophin and Utrophin in Skeletal Muscle of Dystrophinopathies.
Internal Medicine, 1994 Immunostaining of biopsied skeletal muscle of 4 Duchenne (DMD), 12 Becker muscular dystrophy (BMD) and 3 DMD carriers' was performed using monoclonal antibodies against dystrophin and utrophin. In DMD, dystrophin-negative staining was observed except for revertant fibers which showed different stain patterns for each antibody.
Michinari Tashiro +7 more
openaire +4 more sources
Murine obscurin and Obsl1 have functionally redundant roles in sarcolemmal integrity, sarcoplasmic reticulum organization, and muscle metabolism. [PDF]
, 2019 Biological roles of obscurin and its close homolog Obsl1 (obscurin-like 1) have been enigmatic. While obscurin is highly expressed in striated muscles, Obsl1 is found ubiquitously.
Blondelle, Jordan +13 more
core +1 more source
Regulation of branching dynamics by axon-intrinsic asymmetries in Tyrosine Kinase Receptor signaling [PDF]
, 2014 Axonal branching allows a neuron to connect to several targets, increasing neuronal circuit complexity. While axonal branching is well described, the mechanisms that control it remain largely unknown.
De Geest, Natalie +11 more
core +2 more sources
Employment of Microencapsulated Sertoli Cells as a New Tool to Treat Duchenne Muscular Dystrophy
Journal of Functional Morphology and Kinesiology, 2017 Duchenne muscular dystrophy (DMD) is a lethal X-linked pathology due to lack of dystrophin and characterized by progressive muscle degeneration, impaired locomotion and premature death.
Sara Chiappalupi +6 more
doaj +1 more source