Duchenne muscular dystrophy (DMD) is a recessive X-linked form of muscular dystrophy characterized by progressive muscle degeneration. This disease is caused by the mutation or deletion of the dystrophin gene. Currently, there are no effective treatments
Tsubasa Kameyama +20 more
doaj +1 more source
Increased levels of interleukin-6 exacerbate the dystrophic phenotype in mdx mice [PDF]
Duchenne muscular dystrophy (DMD) is characterized by progressive lethal muscle degeneration and chronic inflammatory response. The mdx mouse strain has served as the animal model for human DMD. However, while DMD patients undergo extensive necrosis, the
Berardinelli, Maria Grazia +10 more
core +2 more sources
Cytokine Engineering Approaches for Regenerative Medicine
Engineered cytokines represent a powerful strategy to promote tissue repair and regeneration by precisely modulating immune responses. This review highlights recent advances in cytokine engineering, including strategies to enhance half‐life, improve tissue and cell targeting, and control receptor signaling.
Shiyi Li, Wenhao You, Mikaël M. Martino
wiley +1 more source
Tadalafil Treatment Delays the Onset of Cardiomyopathy in Dystrophin‐Deficient Hearts
BackgroundCardiomyopathy is a leading cause of mortality among Duchenne muscular dystrophy patients and lacks effective therapies. Phosphodiesterase type 5 is implicated in dystrophic pathology, and the phosphodiesterase type 5 inhibitor tadalafil has ...
David W. Hammers +5 more
doaj +1 more source
Role of dystrophin and utrophin for assembly and function of the dystrophin glycoprotein complex in non-muscle tissue [PDF]
.: The dystrophin glycoprotein complex (DGC) is a multimeric protein assembly associated with either the X-linked cytoskeletal protein dystrophin or its autosomal homologue utrophin. In striated muscle cells, the DGC links the extracellular matrix to the
Fritschy, J., Haenggi, T.
core
Evidence for ACTN3 as a genetic modifier of Duchenne muscular dystrophy [PDF]
Duchenne muscular dystrophy (DMD) is characterized by muscle degeneration and progressive weakness. There is considerable inter-patient variability in disease onset and progression, which can confound the results of clinical trials.
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core +2 more sources
High Throughput Screening in Duchenne Muscular Dystrophy: From Drug Discovery to Functional Genomics
Centers for the screening of biologically active compounds and genomic libraries are becoming common in the academic setting and have enabled researchers devoted to developing strategies for the treatment of diseases or interested in studying a ...
Thomas J.J. Gintjee +2 more
doaj +1 more source
Several dystrophin-glycoprotein complex members are present in crude surface membranes but they are sodium dodecyl sulphate invisible in KCl-washed microsomes from mdx mouse muscle. [PDF]
International audienceThe dystrophin-glycoprotein complex (DGC) is a large trans-sarcolemmal complex that provides a linkage between the subsarcolemmal cytoskeleton and the extracellular matrix.
Cherel, Yan +3 more
core +3 more sources
Exercise Increases Utrophin Protein Expression in the MDX Mouse Model of Duchenne Muscular Dystrophy [PDF]
Please view abstract in the attached PDF ...
Gordon, B. S. +2 more
core +1 more source
Role of gelatinases in pathological and physiological processes involving the dystrophin-glycoprotein complex [PDF]
Dystrophin is a cytosolic protein belonging to a membrane-spanning glycoprotein complex, called dystrophin-glycoprotein complex (DGC), that is expressed in many tissues, especially in skeletal muscle and in the nervous system.
Bozzi, Manuela +2 more
core +3 more sources

