Transgenic overexpression of the α7 integrin reduces muscle pathology and improves viability in the dy(W) mouse model of merosin-deficient congenital muscular dystrophy type 1A.

scientific article published on 7 June 2011

Transgenic overexpression of the α7 integrin reduces muscle pathology and improves viability in the dy(W) mouse model of merosin-deficient congenital muscular dystrophy type 1A. is …
instance of (P31):
scholarly articleQ13442814

External links are
P356DOI10.1242/JCS.083311
P932PMC publication ID3113674
P698PubMed publication ID21652631
P5875ResearchGate publication ID51201203

P50authorDean BurkinQ56759871
Ryan D. WuebblesQ64764218
P2093author name stringJachinta E Rooney
Erika T Allred
Jinger A Doe
Margaret Elorza
P2860cites workMutations in the laminin alpha 2-chain gene (LAMA2) cause merosin-deficient congenital muscular dystrophyQ24308817
Mutations in the integrin alpha7 gene cause congenital myopathyQ24317420
Absence of integrin alpha 7 causes a novel form of muscular dystrophyQ24323372
The expanding phenotype of laminin alpha2 chain (merosin) abnormalities: case series and reviewQ24680748
Activation of the lama2 gene in muscle regeneration: abortive regeneration in laminin alpha2-deficiencyQ28141536
Laminin and alpha7beta1 integrin regulate agrin-induced clustering of acetylcholine receptorsQ28141768
Merosin and congenital muscular dystrophyQ28145060
Integrins (alpha7beta1) in muscle function and survival. Disrupted expression in merosin-deficient congenital muscular dystrophyQ28249995
Expression of alpha 7 integrin cytoplasmic domains during skeletal muscle development: alternate forms, conformational change, and homologies with serine/threonine kinases and tyrosine phosphatasesQ28250493
Alternative extracellular and cytoplasmic domains of the integrin alpha 7 subunit are differentially expressed during developmentQ28257104
Altered expression of the alpha7beta1 integrin in human and murine muscular dystrophiesQ28258806
Omigapil ameliorates the pathology of muscle dystrophy caused by laminin-alpha2 deficiencyQ28258826
A new isoform of the laminin receptor integrin alpha 7 beta 1 is developmentally regulated in skeletal muscleQ28261983
Synaptic integrins in developing, adult, and mutant muscle: selective association of alpha1, alpha7A, and alpha7B integrins with the neuromuscular junctionQ28278128
Role for the alpha7beta1 integrin in vascular development and integrityQ28504523
Distribution and function of laminins in the neuromuscular system of developing, adult, and mutant miceQ28593330
Laminin alpha1 chain mediated reduction of laminin alpha2 chain deficient muscular dystrophy involves integrin alpha7beta1 and dystroglycanQ33234933
Role of matrix metalloproteinases in skeletal muscle: migration, differentiation, regeneration and fibrosisQ33575969
The alpha7beta1 integrin in muscle development and diseaseQ33592124
Refinement of the laminin alpha2 chain locus to human chromosome 6q2 in severe and mild merosin deficient congenital muscular dystrophyQ33678375
Molecular basis of muscular dystrophies.Q34044147
Galectin-3: an open-ended storyQ34494555
Laminin isoforms in tumor invasion, angiogenesis and metastasisQ34706652
Transgenic expression of {alpha}7{beta}1 integrin maintains muscle integrity, increases regenerative capacity, promotes hypertrophy, and reduces cardiomyopathy in dystrophic miceQ35083511
The congenital muscular dystrophies in 2004: a century of exciting progressQ35879886
Overexpression of the cytotoxic T cell (CT) carbohydrate inhibits muscular dystrophy in the dyW mouse model of congenital muscular dystrophy 1A.Q35928246
A functional role for specific spliced variants of the alpha7beta1 integrin in acetylcholine receptor clusteringQ36255897
Laminin polymerization induces a receptor-cytoskeleton networkQ36342173
Enhanced expression of the alpha 7 beta 1 integrin reduces muscular dystrophy and restores viability in dystrophic miceQ36381102
The congenital muscular dystrophies: recent advances and molecular insightsQ36681282
Exercise promotes alpha7 integrin gene transcription and protection of skeletal muscleQ36977244
Laminin-111 restores regenerative capacity in a mouse model for alpha7 integrin congenital myopathyQ37073808
Pathology is alleviated by doxycycline in a laminin-alpha2-null model of congenital muscular dystrophyQ37091285
Laminin-111 protein therapy prevents muscle disease in the mdx mouse model for Duchenne muscular dystrophyQ37194805
Merosin-deficient congenital muscular dystrophy. Partial genetic correction in two mouse modelsQ37384623
Inhibition of apoptosis improves outcome in a model of congenital muscular dystrophyQ37625936
Expression and distribution of laminin alpha1 and alpha2 chains in embryonic and adult mouse tissues: an immunochemical approachQ40736493
A synaptic localization domain in the synaptic cleft protein laminin beta 2 (s-laminin)Q41323947
Selective modulation of the interaction of alpha 7 beta 1 integrin with fibronectin and laminin by L-14 lectin during skeletal muscle differentiation.Q41498859
Both laminin and Schwann cell dystroglycan are necessary for proper clustering of sodium channels at nodes of Ranvier.Q41866964
Overexpression of mini-agrin in skeletal muscle increases muscle integrity and regenerative capacity in laminin-alpha2-deficient mice.Q42478963
Feeding problems in merosin deficient congenital muscular dystrophyQ42789487
Galectin-3 mediates the endocytosis of beta-1 integrins by breast carcinoma cellsQ43816780
Alternative splice variants of alpha 7 beta 1 integrin selectively recognize different laminin isoforms.Q43823933
Defective integrin switch and matrix composition at alpha 7-deficient myotendinous junctions precede the onset of muscular dystrophy in mice.Q44316959
Laminin alpha1 chain reduces muscular dystrophy in laminin alpha2 chain deficient miceQ45876189
Merosin-deficient congenital muscular dystrophy: the spectrum of brain involvement on magnetic resonance imagingQ48224297
Expression of laminin alpha1, alpha2, alpha4, and alpha5 chains, fibronectin, and tenascin-C in skeletal muscle of dystrophic 129ReJ dy/dy miceQ48309316
Laminin α2 deficiency and muscular dystrophy; genotype-phenotype correlation in mutant miceQ58108925
Congenital muscular dystrophy with primary laminin alpha2 (merosin) deficiency presenting as inflammatory myopathyQ71864959
Diagnosis and etiology of congenital muscular dystrophyQ80412916
P433issuePt 13
P407language of work or nameEnglishQ1860
P921main subjectpathologyQ7208
congenital muscular dystrophyQ1321884
congenital disorderQ727096
P304page(s)2287-2297
P577publication date2011-06-07
P1433published inJournal of Cell ScienceQ1524177
P1476titleTransgenic overexpression of the α7 integrin reduces muscle pathology and improves viability in the dy(W) mouse model of merosin-deficient congenital muscular dystrophy type 1A.
P478volume124

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cites work (P2860)
Q95840621A Family of Laminin α2 Chain-Deficient Mouse Mutants: Advancing the Research on LAMA2-CMD
Q27322025A splice site mutation in laminin-α2 results in a severe muscular dystrophy and growth abnormalities in zebrafish
Q28283362AAV-mediated overexpression of human α7 integrin leads to histological and functional improvement in dystrophic mice
Q37944530Cell-matrix interactions in muscle disease
Q91906240Current understanding and treatment of cardiac and skeletal muscle pathology in laminin-α2 chain-deficient congenital muscular dystrophy
Q40556372Deletion of integrin α7 subunit does not aggravate the phenotype of laminin α2 chain-deficient mice
Q89624987Distinct cachexia profiles in response to human pancreatic tumours in mouse limb and respiratory muscle
Q47337769Genetic modifiers of Duchenne and facioscapulohumeral muscular dystrophies
Q36608879Genetic overexpression of Serpina3n attenuates muscular dystrophy in mice
Q38882098Impaired fetal muscle development and JAK-STAT activation mark disease onset and progression in a mouse model for merosin-deficient congenital muscular dystrophy
Q33787898Improving Reproducibility of Phenotypic Assessments in the DyW Mouse Model of Laminin-α2 Related Congenital Muscular Dystrophy
Q89963755Laminin and Integrin in LAMA2-Related Congenital Muscular Dystrophy: From Disease to Therapeutics
Q27318580Laminin regulates PDGFRβ(+) cell stemness and muscle development.
Q28087548Laminin therapy for the promotion of muscle regeneration
Q41881081Laminin-111 improves muscle repair in a mouse model of merosin-deficient congenital muscular dystrophy
Q35952091Laminin-111 protein therapy reduces muscle pathology and improves viability of a mouse model of merosin-deficient congenital muscular dystrophy
Q37137768Levels of α7 integrin and laminin-α2 are increased following prednisone treatment in the mdx mouse and GRMD dog models of Duchenne muscular dystrophy
Q47164258Linker proteins restore basement membrane and correct LAMA2-related muscular dystrophy in mice.
Q45016329Mesothelioma cells breaking bad: loss of integrin α7 promotes cell motility and poor clinical outcomes in patients
Q31105806NAD+ biosynthesis ameliorates a zebrafish model of muscular dystrophy
Q30841381Potent pro-inflammatory and pro-fibrotic molecules, osteopontin and galectin-3, are not major disease modulators of laminin α2 chain-deficient muscular dystrophy
Q35201194Quantitative proteomic analysis reveals metabolic alterations, calcium dysregulation, and increased expression of extracellular matrix proteins in laminin α2 chain-deficient muscle
Q92487776Restoring the regenerative balance in neuromuscular disorders: satellite cell activation as therapeutic target in Pompe disease

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