Attenuated muscle regeneration is a key factor in dysferlin-deficient muscular dystrophy.
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Ahnak1 abnormally localizes in muscular dystrophies and contributes to muscle vesicle releaseReduced plasma membrane expression of dysferlin mutants is attributed to accelerated endocytosis via a syntaxin-4-associated pathwayFerlins: regulators of vesicle fusion for auditory neurotransmission, receptor trafficking and membrane repairCharacterization of dysferlin deficient SJL/J mice to assess preclinical drug efficacy: fasudil exacerbates muscle disease phenotypeMembrane wounding triggers ATP release and dysferlin-mediated intercellular calcium signalingDuplication in the microtubule-actin cross-linking factor 1 gene causes a novel neuromuscular condition.Rapid actin-cytoskeleton-dependent recruitment of plasma membrane-derived dysferlin at wounds is critical for muscle membrane repair.Myoferlin regulation by NFAT in muscle injury, regeneration and repair.Extensive mononuclear infiltration and myogenesis characterize recovery of dysferlin-null skeletal muscle from contraction-induced injuries.Proteomic analysis of the dysferlin protein complex unveils its importance for sarcolemmal maintenance and integrityDysferlin forms a dimer mediated by the C2 domains and the transmembrane domain in vitro and in living cells.Myogenesis in dysferlin-deficient myoblasts is inhibited by an intrinsic inflammatory response.Dysferlin-deficient immortalized human myoblasts and myotubes as a useful tool to study dysferlinopathy.New aspects on patients affected by dysferlin deficient muscular dystrophyGenetic ablation of complement C3 attenuates muscle pathology in dysferlin-deficient miceA new distal myopathy with mutation in anoctamin 5.Long-term blocking of calcium channels in mdx mice results in differential effects on heart and skeletal muscleGRAF1 deficiency blunts sarcolemmal injury repair and exacerbates cardiac and skeletal muscle pathology in dystrophin-deficient mice.Dystrophin deficiency exacerbates skeletal muscle pathology in dysferlin-null mice.Upregulated IL-1β in dysferlin-deficient muscle attenuates regeneration by blunting the response to pro-inflammatory macrophagesDysferlin and animal models for dysferlinopathyDiaphragm displays early and progressive functional deficits in dysferlin-deficient mice.Reactive oxygen species generation is not different during isometric and lengthening contractions of mouse muscle.Ferlin proteins in myoblast fusion and muscle growth.Mechanisms of muscle injury, repair, and regeneration.Systemic Delivery of Dysferlin Overlap Vectors Provides Long-Term Gene Expression and Functional Improvement for Dysferlinopathy.Dysferlin rescue by spliceosome-mediated pre-mRNA trans-splicing targeting introns harbouring weakly defined 3' splice sites.Dexamethasone induces dysferlin in myoblasts and enhances their myogenic differentiation.Dysferlin regulates cell adhesion in human monocytes.Dysferlin Binds SNAREs (Soluble N-Ethylmaleimide-sensitive Factor (NSF) Attachment Protein Receptors) and Stimulates Membrane Fusion in a Calcium-sensitive Manner.Muscle membrane repair and inflammatory attack in dysferlinopathy.Impaired muscle growth and response to insulin-like growth factor 1 in dysferlin-mediated muscular dystrophy.Diagnosis by protein analysis of dysferlinopathy in two patients mistaken as polymyositis.Impaired regeneration in calpain-3 null muscle is associated with perturbations in mTORC1 signaling and defective mitochondrial biogenesis.Limited proteolysis as a tool to probe the tertiary conformation of dysferlin and structural consequences of patient missense variant L344P.Plasticity of the Muscle Stem Cell Microenvironment.
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P2860
Attenuated muscle regeneration is a key factor in dysferlin-deficient muscular dystrophy.
description
article científic
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article scientifique
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articolo scientifico
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artigo científico
@pt
bilimsel makale
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scientific article published on 13 March 2009
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vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
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vědecký článek
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name
Attenuated muscle regeneration is a key factor in dysferlin-deficient muscular dystrophy.
@en
Attenuated muscle regeneration is a key factor in dysferlin-deficient muscular dystrophy.
@nl
type
label
Attenuated muscle regeneration is a key factor in dysferlin-deficient muscular dystrophy.
@en
Attenuated muscle regeneration is a key factor in dysferlin-deficient muscular dystrophy.
@nl
prefLabel
Attenuated muscle regeneration is a key factor in dysferlin-deficient muscular dystrophy.
@en
Attenuated muscle regeneration is a key factor in dysferlin-deficient muscular dystrophy.
@nl
P2093
P2860
P356
P1476
Attenuated muscle regeneration is a key factor in dysferlin-deficient muscular dystrophy.
@en
P2093
Kate Bushby
Lars Klinge
Louise H Jørgensen
Mark A Hornsey
Richard Charlton
Rita Barresi
Steven H Laval
Yen-Hui Chiu
P2860
P304
P356
10.1093/HMG/DDP121
P577
2009-03-13T00:00:00Z