Results 51 to 60 of about 21,845 (197)
Cytoskeleton, EarlyView.ABSTRACT We here describe mouse models with complementary homozygous Svil mutations. In skeletal muscle, Svil‐Mut mice express the Svil‐encoded N‐terminus fused to the βgal‐neo gene‐trap tag and lack the highly conserved archvillin C‐terminus; Svil‐KO mice lack expression of all known Svil‐encoded proteins; and Svil‐LoxP mice contain loxP sites for ...
Tara C. Smith +9 more
wiley +1 more source
A novel high-throughput immunofluorescence analysis method for quantifying dystrophin intensity in entire transverse sections of Duchenne muscular dystrophy muscle biopsy samples. [PDF]
PLoS ONE, 2018 Clinical trials using strategies aimed at inducing dystrophin expression in Duchenne muscular dystrophy (DMD) are underway or at advanced planning stage, including splice switching antisense oligonucleotides (AON), drugs to induce read-through of ...
Valentina Sardone +9 more
doaj +1 more source
Dystrophin involvement in peripheral circadian SRF signalling
Life Science Alliance, 2021 Absence of integral sarcolemmal protein, dystrophin, disrupts the RhoA-actin-SRF cascade in skeletal muscle, with subsequent dysregulation of downstream-SRF circadian targets and circadian rhythm.
Corinne A Betts +21 more
doaj +1 more source
Fhod3 in zebrafish supports myofibril stability during growth of embryonic skeletal muscle
Developmental Dynamics, EarlyView.Abstract Background Actin filament organization in cardiomyocytes critically depends on the formin Fhod3, but a role for Fhod3 in skeletal muscle development has not yet been described. Results We demonstrate here that in zebrafish mutated for one of two fhod3 paralog genes, fhod3a, skeletal muscle of the trunk appears normal through 2 days post ...
Aubrie Russell +3 more
wiley +1 more source
Spatiotemporal analysis of dystrophin expression during muscle repair
Skeletal MuscleBackground Dystrophin mRNA is produced from a very large genetic locus and transcription of a single mRNA requires approximately 16 h. This prolonged interval between initiation and completion results in unusual transcriptional behaviour: in skeletal ...
John C.W. Hildyard +3 more
doaj +1 more source
Orphanet Journal of Rare Diseases, 2012 Background Dystrophin is a large essential protein of skeletal and heart muscle. It is a filamentous scaffolding protein with numerous binding domains. Mutations in the DMD gene, which encodes dystrophin, mostly result in the deletion of one or several ...
Nicolas Aurélie +7 more
doaj +1 more source
Three‐dimensional observation of the muscle–tendon integration process in mouse embryos
Developmental Dynamics, EarlyView.Abstract Background Muscle–tendon integration is a crucial step in the morphogenesis of the vertebrate locomotion system. As the limb muscle and tendon progenitor cells derive from distinct embryonic origin, their integration requires precise mutual positioning.
Ramu Sagasaki +10 more
wiley +1 more source
Targeted Exon Skipping to Address “Leaky” Mutations in the Dystrophin Gene
Molecular Therapy: Nucleic Acids, 2012 Protein-truncating mutations in the dystrophin gene lead to the progressive muscle wasting disorder Duchenne muscular dystrophy, whereas in-frame deletions typically manifest as the milder allelic condition, Becker muscular dystrophy.
Sue Fletcher +9 more
doaj +1 more source
A proteomic investigation of forebrain regeneration in the leopard gecko (Eublepharis macularius)
Developmental Dynamics, EarlyView.Our investigation reveals ontogenetic, injury‐ and regeneration‐associated proteomic changes in the leopard gecko forebrain. Abstract Background The ability to replace lost or damaged neurons following an injury is termed reactive neurogenesis. Although reactive neurogenesis has been reported in several lizard species, the molecular mechanisms ...
Alexandra I. Noble +2 more
wiley +1 more source
ESC Heart Failure, Volume 12, Issue 2, Page 1398-1415, April 2025.Abstract Background Cardiac remodelling, a crucial aspect of heart failure, is commonly investigated in preclinical models by quantifying cardiomyocyte cross‐sectional area (CSA) and microvascular density (MVD) via histological methods, such as immunohistochemistry.
Tamás G. Gergely +14 more
wiley +1 more source