Congenital muscular dystrophy: molecular and cellular aspects.
about
Three novel collagen VI chains, alpha4(VI), alpha5(VI), and alpha6(VI)A comparative study of alpha-dystroglycan glycosylation in dystroglycanopathies suggests that the hypoglycosylation of alpha-dystroglycan does not consistently correlate with clinical severityDevelopmental and pathogenic mechanisms of basement membrane assemblyLaminin L4 domain structure resembles adhesion modules in ephrin receptor and other transmembrane glycoproteinsCOL4A1 mutations cause ocular dysgenesis, neuronal localization defects, and myopathy in mice and Walker-Warburg syndrome in humansMaintenance of Hepatic Functions in Primary Human Hepatocytes Cultured on Xeno-Free and Chemical Defined Human Recombinant LamininsExpression of the murine Pomt1 gene in both the developing brain and adult muscle tissues and its relationship with clinical aspects of Walker-Warburg syndromeAberrant alternative splicing and extracellular matrix gene expression in mouse models of myotonic dystrophy.The dystroglycanopathies: the new disorders of O-linked glycosylationRetinal ectopias and mechanically weakened basement membrane in a mouse model of muscle-eye-brain (MEB) disease congenital muscular dystrophyA human-specific deletion in mouse Cmah increases disease severity in the mdx model of Duchenne muscular dystrophyThe Ras antagonist, farnesylthiosalicylic acid (FTS), decreases fibrosis and improves muscle strength in dy/dy mouse model of muscular dystrophy.Zebrafish Fukutin family proteins link the unfolded protein response with dystroglycanopathiesDystromirs as serum biomarkers for monitoring the disease severity in Duchenne muscular Dystrophy.Glycomic analyses of mouse models of congenital muscular dystrophyFrom proteins to genes: immunoanalysis in the diagnosis of muscular dystrophies.Synthetic, structural, and biosynthetic studies of an unusual phospho-glycopeptide derived from α-dystroglycan.Life or death by NFκB, Losartan promotes survival in dy2J/dy2J mouse of MDC1A.Breaches of the pial basement membrane are associated with defective dentate gyrus development in mouse models of congenital muscular dystrophiesOverexpression of the cytotoxic T cell (CT) carbohydrate inhibits muscular dystrophy in the dyW mouse model of congenital muscular dystrophy 1A.Mesoangioblast delivery of miniagrin ameliorates murine model of merosin-deficient congenital muscular dystrophy type 1A.Developmental expression of the neuron-specific N-acetylglucosaminyltransferase Vb (GnT-Vb/IX) and identification of its in vivo glycan products in comparison with those of its paralog, GnT-VUnderstanding the importance of selenium and selenoproteins in muscle functionUpregulation of COL6A1 is predictive of poor prognosis in clear cell renal cell carcinoma patientsMechanisms of disease: congenital muscular dystrophies-glycosylation takes center stage.Conditional knockout of protein O-mannosyltransferase 2 reveals tissue-specific roles of O-mannosyl glycosylation in brain development.Biochemical and biophysical changes underlie the mechanisms of basement membrane disruptions in a mouse model of dystroglycanopathy.Laminin-111 restores regenerative capacity in a mouse model for alpha7 integrin congenital myopathyMuscular dystrophy associated with alpha-dystroglycan deficiency in Sphynx and Devon Rex cats.Muscle mitochondrial uncoupling dismantles neuromuscular junction and triggers distal degeneration of motor neurons.Congenital muscular dystrophy. Part I: a review of phenotypical and diagnostic aspects.Congenital muscular dystrophy. Part II: a review of pathogenesis and therapeutic perspectives.Basement membrane components are key players in specialized extracellular matrices.Golgi glycosylation and human inherited diseases.Cell-matrix interactions in muscle disease.Chimeric protein repair of laminin polymerization ameliorates muscular dystrophy phenotype.The nature and biology of basement membranes.B4GALNT2 (GALGT2) Gene Therapy Reduces Skeletal Muscle Pathology in the FKRP P448L Mouse Model of Limb Girdle Muscular Dystrophy 2I.Diagnosis and etiology of congenital muscular dystrophy: We are halfway there.RPTPζ/phosphacan is abnormally glycosylated in a model of muscle-eye-brain disease lacking functional POMGnT1
P2860
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P2860
Congenital muscular dystrophy: molecular and cellular aspects.
description
2005 nî lūn-bûn
@nan
2005年の論文
@ja
2005年論文
@yue
2005年論文
@zh-hant
2005年論文
@zh-hk
2005年論文
@zh-mo
2005年論文
@zh-tw
2005年论文
@wuu
2005年论文
@zh
2005年论文
@zh-cn
name
Congenital muscular dystrophy: molecular and cellular aspects.
@ast
Congenital muscular dystrophy: molecular and cellular aspects.
@en
type
label
Congenital muscular dystrophy: molecular and cellular aspects.
@ast
Congenital muscular dystrophy: molecular and cellular aspects.
@en
prefLabel
Congenital muscular dystrophy: molecular and cellular aspects.
@ast
Congenital muscular dystrophy: molecular and cellular aspects.
@en
P1476
Congenital muscular dystrophy: molecular and cellular aspects
@en
P2093
P2888
P304
P356
10.1007/S00018-004-4510-4
P577
2005-04-01T00:00:00Z