Improving Reproducibility of Phenotypic Assessments in the DyW Mouse Model of Laminin-α2 Related Congenital Muscular Dystrophy

scientific article

Improving Reproducibility of Phenotypic Assessments in the DyW Mouse Model of Laminin-α2 Related Congenital Muscular Dystrophy is …
instance of (P31):
scholarly articleQ13442814
review articleQ7318358

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P356DOI10.3233/JND-170217
P8608Fatcat IDrelease_g42kftf4ibhphah3fbfjj2aojy
P932PMC publication ID5467719
P698PubMed publication ID28550268

P50authorKanneboyina NagarajuQ27662260
Dean BurkinQ56759871
Heather Gordish-DressmanQ57474854
P2093author name stringRaffaella Willmann
Qing Yu
Markus A Rüegg
Anne Rutkowski
Mahasweta Girgenrath
Pam M Van Ry
Cathleen Lutz
Vivian Cruz
Ayar Kumar
Caroline B M Coffey
Laurent Bogdanik
Sarina Meinen
P2860cites workMutations in the laminin alpha 2-chain gene (LAMA2) cause merosin-deficient congenital muscular dystrophyQ24308817
Apoptosis inhibitors and mini-agrin have additive benefits in congenital muscular dystrophy miceQ24602606
Prevalence of congenital muscular dystrophy in Italy: a population studyQ28256516
Omigapil ameliorates the pathology of muscle dystrophy caused by laminin-alpha2 deficiencyQ28258826
Merosin, a protein specific for basement membranes of Schwann cells, striated muscle, and trophoblast, is expressed late in nerve and muscle developmentQ33557296
An agrin minigene rescues dystrophic symptoms in a mouse model for congenital muscular dystrophy.Q34091913
Promotion of plasma membrane repair by vitamin E.Q35639232
Pathology is alleviated by doxycycline in a laminin-alpha2-null model of congenital muscular dystrophyQ37091285
Merosin-deficient congenital muscular dystrophy. Partial genetic correction in two mouse modelsQ37384623
Consensus statement on standard of care for congenital muscular dystrophiesQ37552615
Inhibition of apoptosis improves outcome in a model of congenital muscular dystrophyQ37625936
Mitochondria as possible pharmaceutical targets for the effects of vitamin E and its homologues in oxidative stress-related diseasesQ37903067
Disruption of the lama2 gene in embryonic stem cells: laminin alpha 2 is necessary for sustenance of mature muscle cellsQ41032544
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.Q42489627
Inhibiting TGF-β activity improves respiratory function in mdx miceQ42732727
Impaired respiratory function in mdx and mdx/utrn(+/-) miceQ42741029
Histopathologic findings in the sacrocaudalis dorsalis medialis muscle of horses with vitamin E-responsive muscle atrophy and weaknessQ45006054
Congenital muscular dystrophy with merosin deficiencyQ59281293
Genotype–phenotype correlation in a large population of muscular dystrophy patients with LAMA2 mutationsQ61794269
Functional deterioration and selenium-vitamin E treatment in myotonic dystrophy. A placebo-controlled studyQ72274091
Merosin-deficient congenital muscular dystrophy, autosomal recessive (MDC1A, MIM#156225, LAMA2 gene coding for alpha2 chain of laminin)Q77894566
P275copyright licenseCreative Commons Attribution-NonCommercial 4.0 InternationalQ34179348
P433issue2
P921main subjectmembrane proteinQ423042
congenital disorderQ727096
muscular dystrophyQ1137767
congenital muscular dystrophyQ1321884
reproducibilityQ1425625
fibrous proteinQ1976174
animal disease modelQ64732998
biomedical investigative techniqueQ66648976
P304page(s)115-126
P577publication date2017-05-24
P1433published inJournal of neuromuscular diseasesQ27726242
P1476titleImproving Reproducibility of Phenotypic Assessments in the DyW Mouse Model of Laminin-α2 Related Congenital Muscular Dystrophy
P478volume4

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cites work (P2860)
Q95840621A Family of Laminin α2 Chain-Deficient Mouse Mutants: Advancing the Research on LAMA2-CMD
Q98464776Brain Dysfunction in LAMA2-Related Congenital Muscular Dystrophy: Lessons From Human Case Reports and Mouse Models
Q91906240Current understanding and treatment of cardiac and skeletal muscle pathology in laminin-α2 chain-deficient congenital muscular dystrophy
Q47345657Laminin-deficient muscular dystrophy: Molecular pathogenesis and structural repair strategies.
Q90733720Mouse models for muscular dystrophies: an overview

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