Dysferlin associates with the developing T-tubule system in rodent and human skeletal muscle
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Alternate splicing of dysferlin C2A confers Ca²⁺-dependent and Ca²⁺-independent binding for membrane repairDysferlin at transverse tubules regulates Ca(2+) homeostasis in skeletal muscleDefective membrane remodeling in neuromuscular diseases: insights from animal modelsFerlins: regulators of vesicle fusion for auditory neurotransmission, receptor trafficking and membrane repairLack of correlation between outcomes of membrane repair assay and correction of dystrophic changes in experimental therapeutic strategy in dysferlinopathyMembrane damage-induced vesicle-vesicle fusion of dysferlin-containing vesicles in muscle cells requires microtubules and kinesin.An actin-dependent annexin complex mediates plasma membrane repair in muscle.Dysferlin interacts with histone deacetylase 6 and increases alpha-tubulin acetylationContribution of dysferlin deficiency to skeletal muscle pathology in asymptomatic and severe dystroglycanopathy models: generation of a new model for Fukuyama congenital muscular dystrophyNew aspects on patients affected by dysferlin deficient muscular dystrophyModular dispensability of dysferlin C2 domains reveals rational design for mini-dysferlin molecules.Altered splicing of the BIN1 muscle-specific exon in humans and dogs with highly progressive centronuclear myopathyFrom proteins to genes: immunoanalysis in the diagnosis of muscular dystrophies.T-tubule biogenesis and triad formation in skeletal muscle and implication in human diseases.Unmasking potential intracellular roles for dysferlin through improved immunolabeling methodsGRAF1 deficiency blunts sarcolemmal injury repair and exacerbates cardiac and skeletal muscle pathology in dystrophin-deficient mice.Eps 15 Homology Domain (EHD)-1 Remodels Transverse Tubules in Skeletal Muscle.The absence of dysferlin induces the expression of functional connexin-based hemichannels in human myotubesDysferlin and animal models for dysferlinopathyMembrane fusion in muscle development and repair.Plasma Membrane Repair in Health and DiseaseAnimal models of muscular dystrophyJunctophilin-2 is necessary for T-tubule maturation during mouse heart development.Dysferlin stabilizes stress-induced Ca2+ signaling in the transverse tubule membrane.Dysferlin and myoferlin regulate transverse tubule formation and glycerol sensitivity.Dynamin 2 the rescue for centronuclear myopathy.Translational research and therapeutic perspectives in dysferlinopathies.Ferlin proteins in myoblast fusion and muscle growth.Dysferlin-deficient muscular dystrophy and innate immune activation.Ultrastructural myopathology in the molecular era.EHD1 mediates vesicle trafficking required for normal muscle growth and transverse tubule development.Dysferlin rescue by spliceosome-mediated pre-mRNA trans-splicing targeting introns harbouring weakly defined 3' splice sites.MicroRNA signatures predict dysregulated vitamin D receptor and calcium pathways status in limb girdle muscle dystrophies (LGMD) 2A/2B.A comparison of AAV strategies distinguishes overlapping vectors for efficient systemic delivery of the 6.2 kb Dysferlin coding sequenceA novel dysferlin mutant pseudoexon bypassed with antisense oligonucleotides.Muscle membrane repair and inflammatory attack in dysferlinopathy.A naturally occurring human minidysferlin protein repairs sarcolemmal lesions in a mouse model of dysferlinopathy.Amphiphysin (BIN1) negatively regulates dynamin 2 for normal muscle maturation.Treatment with Recombinant Human MG53 Protein Increases Membrane Integrity in a Mouse Model of Limb Girdle Muscular Dystrophy 2B.Tubular Aggregates and Cylindrical Spirals Have Distinct Immunohistochemical Signatures.
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Dysferlin associates with the developing T-tubule system in rodent and human skeletal muscle
description
article
@en
wetenschappelijk artikel
@nl
наукова стаття, опублікована в лютому 2010
@uk
name
Dysferlin associates with the ...... dent and human skeletal muscle
@en
Dysferlin associates with the ...... dent and human skeletal muscle
@nl
type
label
Dysferlin associates with the ...... dent and human skeletal muscle
@en
Dysferlin associates with the ...... dent and human skeletal muscle
@nl
prefLabel
Dysferlin associates with the ...... dent and human skeletal muscle
@en
Dysferlin associates with the ...... dent and human skeletal muscle
@nl
P2093
P2860
P356
P1433
P1476
Dysferlin associates with the ...... dent and human skeletal muscle
@en
P2093
Caroline Sewry
John Harris
Kate Bushby
Lars Klinge
Louise Anderson
Mark Hornsey
Richard Charlton
Rita Barresi
Steve Laval
Yen-Hui Chiu
P2860
P304
P356
10.1002/MUS.21166
P577
2010-02-01T00:00:00Z