Merosin-deficient congenital muscular dystrophy. Partial genetic correction in two mouse models
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Skeletal muscle laminin and MDC1A: pathogenesis and treatment strategies.Form and function: the laminin family of heterotrimersApoptosis inhibitors and mini-agrin have additive benefits in congenital muscular dystrophy miceDiseased muscles that lack dystrophin or laminin-alpha2 have altered compositions and proliferation of mononuclear cell populationsLaminin regulates PDGFRβ(+) cell stemness and muscle development.Distinct roles for laminin globular domains in laminin alpha1 chain mediated rescue of murine laminin alpha2 chain deficiencySarcoglycan isoforms in skeletal muscleMagnetic Resonance Imaging Is Sensitive to Pathological Amelioration in a Model for Laminin-Deficient Congenital Muscular Dystrophy (MDC1A)Mesenchymal stem cells facilitate axon sorting, myelination, and functional recovery in paralyzed mice deficient in Schwann cell-derived laminin.Laminin alpha1 chain mediated reduction of laminin alpha2 chain deficient muscular dystrophy involves integrin alpha7beta1 and dystroglycan.Identification of alpha-dystroglycan binding sequences in the laminin alpha2 chain LG4-5 moduleImproving Reproducibility of Phenotypic Assessments in the DyW Mouse Model of Laminin-α2 Related Congenital Muscular Dystrophy.Defining the role of laminin-332 in carcinoma.Amelioration of laminin-alpha2-deficient congenital muscular dystrophy by somatic gene transfer of miniagrin.Bcl-2 inhibits the innate immune response during early pathogenesis of murine congenital muscular dystrophyLaminins of the neuromuscular system.Laminins and human disease.Transgenic isolation of skeletal muscle and kidney defects in laminin beta2 mutant mice: implications for Pierson syndromeOmigapil treatment decreases fibrosis and improves respiratory rate in dy(2J) mouse model of congenital muscular dystrophyPeripheral nerve pathology, including aberrant Schwann cell differentiation, is ameliorated by doxycycline in a laminin-α2-deficient mouse model of congenital muscular dystrophy.Elimination of myostatin does not combat muscular dystrophy in dy mice but increases postnatal lethality.Quantitative proteomic analysis reveals metabolic alterations, calcium dysregulation, and increased expression of extracellular matrix proteins in laminin α2 chain-deficient muscle.Proinflammatory signals and the loss of lymphatic vessel hyaluronan receptor-1 (LYVE-1) in the early pathogenesis of laminin alpha2-deficient skeletal muscle.Laminin: the crux of basement membrane assembly.ADAM12 alleviates the skeletal muscle pathology in mdx dystrophic mice.The muscular dystrophies: from genes to therapies.Overexpression of the cytotoxic T cell (CT) carbohydrate inhibits muscular dystrophy in the dyW mouse model of congenital muscular dystrophy 1A.Laminin-111 protein therapy reduces muscle pathology and improves viability of a mouse model of merosin-deficient congenital muscular dystrophyMesoangioblast delivery of miniagrin ameliorates murine model of merosin-deficient congenital muscular dystrophy type 1A.Linker molecules between laminins and dystroglycan ameliorate laminin-alpha2-deficient muscular dystrophy at all disease stagesFunctional sites in the laminin alpha chains.Conditional disruption of beta 1 integrin in Schwann cells impedes interactions with axons.Laminin gamma1 is critical for Schwann cell differentiation, axon myelination, and regeneration in the peripheral nerveDeletion of murine SMN exon 7 directed to skeletal muscle leads to severe muscular dystrophyLaminin-211 in skeletal muscle function.Long-term survival of transplanted stem cells in immunocompetent mice with muscular dystrophy.Animal models of muscular dystrophyLaminin-111 restores regenerative capacity in a mouse model for alpha7 integrin congenital myopathyPathology is alleviated by doxycycline in a laminin-alpha2-null model of congenital muscular dystrophyGenetic and pharmacologic inhibition of mitochondrial-dependent necrosis attenuates muscular dystrophy.
P2860
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P2860
Merosin-deficient congenital muscular dystrophy. Partial genetic correction in two mouse models
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
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scientific article published on August 1998
@en
vedecký článok
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vetenskaplig artikel
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videnskabelig artikel
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vědecký článek
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name
Merosin-deficient congenital m ...... correction in two mouse models
@en
Merosin-deficient congenital m ...... orrection in two mouse models.
@nl
type
label
Merosin-deficient congenital m ...... correction in two mouse models
@en
Merosin-deficient congenital m ...... orrection in two mouse models.
@nl
prefLabel
Merosin-deficient congenital m ...... correction in two mouse models
@en
Merosin-deficient congenital m ...... orrection in two mouse models.
@nl
P2093
P2860
P356
P1476
Merosin-deficient congenital m ...... correction in two mouse models
@en
P2093
P2860
P304
P356
10.1172/JCI3705
P407
P577
1998-08-01T00:00:00Z