Lack of correlation between outcomes of membrane repair assay and correction of dystrophic changes in experimental therapeutic strategy in dysferlinopathy
about
Alternate splicing of dysferlin C2A confers Ca²⁺-dependent and Ca²⁺-independent binding for membrane repairBone marrow transplantation in dysferlin-deficient mice results in a mild functional improvement.Modular dispensability of dysferlin C2 domains reveals rational design for mini-dysferlin molecules.Myofiber damage precedes macrophage infiltration after in vivo injury in dysferlin-deficient A/J mouse skeletal muscle.Enhanced Muscular Dystrophy from Loss of Dysferlin Is Accompanied by Impaired Annexin A6 Translocation after Sarcolemmal Disruption.The absence of dysferlin induces the expression of functional connexin-based hemichannels in human myotubesMembrane Repair: Mechanisms and Pathophysiology.Plasma Membrane Repair: A Central Process for Maintaining Cellular Homeostasis.Membrane fusion in muscle development and repair.Plasma Membrane Repair in Health and DiseaseProspect of gene therapy for cardiomyopathy in hereditary muscular dystrophy.FER-1/Dysferlin promotes cholinergic signaling at the neuromuscular junction in C. elegans and miceDysferlin stabilizes stress-induced Ca2+ signaling in the transverse tubule membrane.Dysferlin and myoferlin regulate transverse tubule formation and glycerol sensitivity.Dysferlin-deficient muscular dystrophy and innate immune activation.Systemic Delivery of Dysferlin Overlap Vectors Provides Long-Term Gene Expression and Functional Improvement for Dysferlinopathy.Structure-Based Designed Nano-Dysferlin Significantly Improves Dysferlinopathy in BLA/J Mice.Enzymatic cleavage of myoferlin releases a dual C2-domain module linked to ERK signalling.Dysferlin rescue by spliceosome-mediated pre-mRNA trans-splicing targeting introns harbouring weakly defined 3' splice sites.Cis-splicing and translation of the pre-trans-splicing molecule combine with efficiency in spliceosome-mediated RNA trans-splicingPlasma membrane wounding and repair in pulmonary diseases.Muscle cell communication in development and repair.A comparison of AAV strategies distinguishes overlapping vectors for efficient systemic delivery of the 6.2 kb Dysferlin coding sequenceDysferlin Binds SNAREs (Soluble N-Ethylmaleimide-sensitive Factor (NSF) Attachment Protein Receptors) and Stimulates Membrane Fusion in a Calcium-sensitive Manner.Full-length dysferlin expression driven by engineered human dystrophic blood derived CD133+ stem cells.Treatment with Recombinant Human MG53 Protein Increases Membrane Integrity in a Mouse Model of Limb Girdle Muscular Dystrophy 2B.Effect of Ibuprofen on Skeletal Muscle of Dysferlin-null Mice.Serum exosomes can restore cellular function in vitro and be used for diagnosis in dysferlinopathy.Diltiazem improves contractile properties of skeletal muscle in dysferlin-deficient BLAJ mice, but does not reduce contraction-induced muscle damage.
P2860
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P2860
Lack of correlation between outcomes of membrane repair assay and correction of dystrophic changes in experimental therapeutic strategy in dysferlinopathy
description
2012 nî lūn-bûn
@nan
2012 թուականին հրատարակուած գիտական յօդուած
@hyw
2012 թվականին հրատարակված գիտական հոդված
@hy
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
name
Lack of correlation between ou ...... ic strategy in dysferlinopathy
@ast
Lack of correlation between ou ...... ic strategy in dysferlinopathy
@en
Lack of correlation between ou ...... ic strategy in dysferlinopathy
@nl
type
label
Lack of correlation between ou ...... ic strategy in dysferlinopathy
@ast
Lack of correlation between ou ...... ic strategy in dysferlinopathy
@en
Lack of correlation between ou ...... ic strategy in dysferlinopathy
@nl
prefLabel
Lack of correlation between ou ...... ic strategy in dysferlinopathy
@ast
Lack of correlation between ou ...... ic strategy in dysferlinopathy
@en
Lack of correlation between ou ...... ic strategy in dysferlinopathy
@nl
P2093
P2860
P50
P3181
P1433
P1476
Lack of correlation between ou ...... ic strategy in dysferlinopathy
@en
P2093
Carinne Roudaut
Daniel Stockholm
Isabelle Richard
Laurence Suel
Marina Pryadkina
Martin Krahn
Nathalie Bourg
Perrine Borel
Robert J Bloch
Rumaisa Bashir
P2860
P304
P3181
P356
10.1371/JOURNAL.PONE.0038036
P407
P577
2012-05-29T00:00:00Z