Linker molecules between laminins and dystroglycan ameliorate laminin-alpha2-deficient muscular dystrophy at all disease stages
about
Skeletal muscle laminin and MDC1A: pathogenesis and treatment strategies.Apoptosis inhibitors and mini-agrin have additive benefits in congenital muscular dystrophy miceDevelopmental and pathogenic mechanisms of basement membrane assemblyLaminin regulates PDGFRβ(+) cell stemness and muscle development.Distinct roles for laminin globular domains in laminin alpha1 chain mediated rescue of murine laminin alpha2 chain deficiencyMuscle-wide secretion of a miniaturized form of neural agrin rescues focal neuromuscular innervation in agrin mutant mice.Muscle-specific expression of insulin-like growth factor 1 improves outcome in Lama2Dy-w mice, a model for congenital muscular dystrophy type 1APeripheral nerve pathology, including aberrant Schwann cell differentiation, is ameliorated by doxycycline in a laminin-α2-deficient mouse model of congenital muscular dystrophy.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-α1 LG4-5 domain binding to dystroglycan mediates muscle cell survival, growth, and the AP-1 and NF-κB transcription factors but also has adverse effects.Meeting Report: New Directions in the Biology and Disease of Skeletal Muscle 2014Overexpression 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.Dietary fish oil supplementation inhibits formation of endometriosis-associated adhesions in a chimeric mouse modelDiverse cell signaling events modulated by perlecan.Pathology is alleviated by doxycycline in a laminin-alpha2-null model of congenital muscular dystrophyScaffold-forming and Adhesive Contributions of Synthetic Laminin-binding Proteins to Basement Membrane AssemblyIdentification of an agrin mutation that causes congenital myasthenia and affects synapse function.Congenital muscular dystrophy. Part II: a review of pathogenesis and therapeutic perspectives.Laminin: loss-of-function studies.Angiotensin II type 1 receptor antagonists alleviate muscle pathology in the mouse model for laminin-α2-deficient congenital muscular dystrophy (MDC1A).Laminin-111 improves muscle repair in a mouse model of merosin-deficient congenital muscular dystrophy.Lkb1 is indispensable for skeletal muscle development, regeneration, and satellite cell homeostasisDystroglycan and dystroglycanopathies: report of the 187th ENMC Workshop 11-13 November 2011, Naarden, The NetherlandsLG4-5 domains of laminin-211 binds alpha-dystroglycan to allow myotube attachment and prevent anoikis.The UPF3B gene, implicated in intellectual disability, autism, ADHD and childhood onset schizophrenia regulates neural progenitor cell behaviour and neuronal outgrowth.Linker proteins restore basement membrane and correct LAMA2-related muscular dystrophy in mice.Pathogenicity of a Human Laminin β2 Mutation Revealed in Models of Alport Syndrome.Laminin-deficient muscular dystrophy: Molecular pathogenesis and structural repair strategies.Muscular dystrophy meets protein biochemistry, the mother of invention.Proteasome inhibition improves the muscle of laminin α2 chain-deficient mice.Autophagy is increased in laminin α2 chain-deficient muscle and its inhibition improves muscle morphology in a mouse model of MDC1A.
P2860
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P2860
Linker molecules between laminins and dystroglycan ameliorate laminin-alpha2-deficient muscular dystrophy at all disease stages
description
2007 nî lūn-bûn
@nan
2007年の論文
@ja
2007年論文
@yue
2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
@zh-tw
2007年论文
@wuu
2007年论文
@zh
2007年论文
@zh-cn
name
Linker molecules between lamin ...... ystrophy at all disease stages
@ast
Linker molecules between lamin ...... ystrophy at all disease stages
@en
type
label
Linker molecules between lamin ...... ystrophy at all disease stages
@ast
Linker molecules between lamin ...... ystrophy at all disease stages
@en
prefLabel
Linker molecules between lamin ...... ystrophy at all disease stages
@ast
Linker molecules between lamin ...... ystrophy at all disease stages
@en
P2093
P2860
P356
P1476
Linker molecules between lamin ...... ystrophy at all disease stages
@en
P2093
Markus A Ruegg
Patrizia Barzaghi
Sarina Meinen
P2860
P304
P356
10.1083/JCB.200611152
P407
P577
2007-03-01T00:00:00Z