about
Nonhuman genetics. Genomic basis for the convergent evolution of electric organsSwimming into prominence: the zebrafish as a valuable tool for studying human myopathies and muscular dystrophies.A TALEN-Exon Skipping Design for a Bethlem Myopathy Model in ZebrafishRemodeling of Mitochondrial Flashes in Muscular Development and Dystrophy in ZebrafishHanging on for the ride: adhesion to the extracellular matrix mediates cellular responses in skeletal muscle morphogenesis and diseaseRecent advances using zebrafish animal models for muscle disease drug discoveryLeiomodin-3 dysfunction results in thin filament disorganization and nemaline myopathy.Neb: a zebrafish model of nemaline myopathy due to nebulin mutation.Mitochondrial dysfunction and defective autophagy in the pathogenesis of collagen VI muscular dystrophies.Modifications to toxic CUG RNAs induce structural stability, rescue mis-splicing in a myotonic dystrophy cell model and reduce toxicity in a myotonic dystrophy zebrafish model.Melanocytes from Patients Affected by Ullrich Congenital Muscular Dystrophy and Bethlem Myopathy have Dysfunctional Mitochondria That Can be Rescued with Cyclophilin Inhibitors.Zebrafish based small molecule screens for novel DMD drugs.Homozygous splice mutation in CWF19L1 in a Turkish family with recessive ataxia syndromeColVI myopathies: where do we stand, where do we go?Expression of collagen VI α5 and α6 chains in human muscle and in Duchenne muscular dystrophy-related muscle fibrosis.Critical evaluation of the use of cell cultures for inclusion in clinical trials of patients affected by collagen VI myopathiesDiscovery, Synthesis, and Optimization of Diarylisoxazole-3-carboxamides as Potent Inhibitors of the Mitochondrial Permeability Transition Pore.Zebrafish models flex their muscles to shed light on muscular dystrophies.Animal models of muscular dystrophySarcolemmal specific collagen VI deficient myopathy in a Labrador RetrieverTranscriptional changes and developmental abnormalities in a zebrafish model of myotonic dystrophy type 1.212th ENMC International Workshop: Animal models of congenital muscular dystrophies, Naarden, The Netherlands, 29-31 May 2015.Annexin A2 mediates secretion of collagen VI, pulmonary elasticity and apoptosis of bronchial epithelial cells.Ultrastructural changes in muscle cells of patients with collagen VI-related myopathies.Zebrafish embryos and larvae: a new generation of disease models and drug screens.Shutting down the pore: The search for small molecule inhibitors of the mitochondrial permeability transition.Tendon Extracellular Matrix Alterations in Ullrich Congenital Muscular Dystrophy.Two novel COLVI long chains in zebrafish that are essential for muscle development.Matrilin-1 is essential for zebrafish development by facilitating collagen II secretionMYBPC1 mutations impair skeletal muscle function in zebrafish models of arthrogryposisRepression of phosphatidylinositol transfer protein α ameliorates the pathology of Duchenne muscular dystrophy.Cartilage ultrastructure in proteoglycan-deficient zebrafish mutants brings to light new candidate genes for human skeletal disorders.NIM811, a cyclophilin inhibitor without immunosuppressive activity, is beneficial in collagen VI congenital muscular dystrophy models.It is all about the support - The role of the extracellular matrix in regenerating axon guidance.Treating pediatric neuromuscular disorders: The future is now.[Potential of the zebrafish model to study congenital muscular dystrophies].Collagen VI in healthy and diseased nervous system.
P2860
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P2860
description
2010 nî lūn-bûn
@nan
2010 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի մարտին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
Zebrafish models of collagen VI-related myopathies
@ast
Zebrafish models of collagen VI-related myopathies
@en
type
label
Zebrafish models of collagen VI-related myopathies
@ast
Zebrafish models of collagen VI-related myopathies
@en
prefLabel
Zebrafish models of collagen VI-related myopathies
@ast
Zebrafish models of collagen VI-related myopathies
@en
P2093
P2860
P356
P1476
Zebrafish models of collagen VI-related myopathies
@en
P2093
C G Bonnemann
J J Dowling
W R Telfer
P2860
P304
P356
10.1093/HMG/DDQ126
P577
2010-03-25T00:00:00Z