Results 31 to 40 of about 23,636 (243)

Potent Dystrophin knock-Down in Vitro and in Vivo Using RNAi Technonlogy and Expression Signature of Myotubes with Dystrophin knocked Down: Attempts at Unravelling the Mystery [PDF]

open access: yesIranian Journal of Public Health, 2005
Duchenne Muscular Dystrophy (DMD) is one of a group of genetically heterogeneous muscular dystrophies that are characterized by progressive weakness and wasting of skeletal muscle.
MM Ghahramani Seno   +6 more
doaj   +1 more source

Optimized lentiviral vector to restore full-length dystrophin via a cell-mediated approach in a mouse model of Duchenne muscular dystrophy

open access: yesMolecular Therapy: Methods & Clinical Development, 2022
Duchenne muscular dystrophy (DMD) is a muscle wasting disorder caused by mutations in the DMD gene. Restoration of full-length dystrophin protein in skeletal muscle would have therapeutic benefit, but lentivirally mediated delivery of such a large gene ...
Jinhong Meng   +5 more
doaj  

When Size Really Matters: The Eccentricities of Dystrophin Transcription and the Hazards of Quantifying mRNA from Very Long Genes

open access: yesBiomedicines, 2023
At 2.3 megabases in length, the dystrophin gene is enormous: transcription of a single mRNA requires approximately 16 h. Principally expressed in skeletal muscle, the dystrophin protein product protects the muscle sarcolemma against contraction-induced ...
John C. W. Hildyard, Richard J. Piercy
doaj   +1 more source

Perspective: Spectrin-Like Repeats in Dystrophin Have Unique Binding Preferences for Syntrophin Adaptors That Explain the Mystery of How nNOSμ Localizes to the Sarcolemma

open access: yesFrontiers in Physiology, 2018
Dystrophin is a massive multi-domain protein composed of specialized amino and carboxyl termini that are separated by 24 spectrin-like repeats. Dystrophin performs critical structural and signaling roles that are indispensable for the functional ...
Justin M. Percival
doaj   +1 more source

Dystrophin gene expression and intracellular calcium changes in the giant freshwater prawn, Macrobrachium rosenbergii, in response to white spot symptom disease infection

open access: yesHeliyon, 2017
Background: Dystrophin, an essential protein functional in the maintenance of muscle structural integrity is known to be responsible for muscle deterioration during white spot syndrome virus (WSSV) infection among prawn species.
Anees Fathima Noor   +5 more
doaj   +1 more source

Low human dystrophin levels prevent cardiac electrophysiological and structural remodelling in a Duchenne mouse model

open access: yesScientific Reports, 2021
Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disorder caused by loss of dystrophin. This lack also affects cardiac structure and function, and cardiovascular complications are a major cause of death in DMD.
Gerard A. Marchal   +8 more
doaj   +1 more source

Utilization of an Antibody Specific for Human Dystrophin to Follow Myoblast Transplantation in Nude Mice

open access: yesCell Transplantation, 1993
Human myoblasts were transplanted in nude mice. The efficacy of these transplantations was analyzed using a monoclonal antibody (NCLDys3) specific for human dystrophin. This antibody did not reveal any dystrophin in nude mice that did not receive a human
Johnny Huard   +4 more
doaj   +1 more source

Characterization of 65 epitope-specific dystrophin monoclonal antibodies in canine and murine models of duchenne muscular dystrophy by immunostaining and western blot. [PDF]

open access: yesPLoS ONE, 2014
Epitope-specific monoclonal antibodies can provide unique insights for studying cellular proteins. Dystrophin is one of the largest cytoskeleton proteins encoded by 79 exons. The absence of dystrophin results in Duchenne muscular dystrophy (DMD).
Kasun Kodippili   +6 more
doaj   +1 more source

The interaction of actin with dystrophin

open access: yesFEBS Letters, 1990
Proton NMR spectroscopy of synthetic peptides corresponding to defined regions of human dystrophin has been employed to study the interaction with F‐actin. No evidence of interaction with a C‐terminal region corresponding to amino acid residues 3429–3440 was obtained.
Barry A. Levine   +3 more
openaire   +3 more sources

Lentiviral vectors can be used for full-length dystrophin gene therapy

open access: yesScientific 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
doaj   +1 more source

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