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

Willmann, Raffaella and Gordish-Dressman, Heather and Meinen, Sarina and Rüegg, Markus A. and Yu, Qing and Nagaraju, Kanneboyina and Kumar, Ayar and Girgenrath, Mahasweta and Coffey, Caroline B. M. and Cruz, Vivian and Van Ry, Pam M. and Bogdanik, Laurent and Lutz, Cathleen and Rutkowski, Anne and Burkin, Dean J.. (2017) Improving Reproducibility of Phenotypic Assessments in the DyW Mouse Model of Laminin-α2 Related Congenital Muscular Dystrophy. Journal of Neuromuscular Diseases, 4 (2). pp. 115-126.

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Abstract

Laminin-α2 related Congenital Muscular Dystrophy (LAMA2-CMD) is a progressive muscle disease caused by partial or complete deficiency of laminin-211, a skeletal muscle extracellular matrix protein. In the last decade, basic science research has queried underlying disease mechanisms in existing LAMA2-CMD murine models and identified possible clinical targets and pharmacological interventions. Experimental rigor in preclinical studies is critical to efficiently and accurately quantify both negative and positive results, degree of efficiency of potential therapeutics and determine whether to move a compound forward for additional preclinical testing. In this review, we compare published available data measured to assess three common parameters in the widely used mouse model DyW, that mimics LAMA2-CMD, we quantify variability and analyse its possible sources. Finally, on the basis of this analysis, we suggest standard set of assessments and the use of available standardized protocols, to reduce variability of outcomes in the future and to improve the value of preclinical research.
Faculties and Departments:05 Faculty of Science > Departement Biozentrum > Neurobiology > Pharmacology/Neurobiology (Rüegg)
UniBasel Contributors:Rüegg, Markus A.
Item Type:Article, refereed
Article Subtype:Research Article
Publisher:IOS Press
ISSN:2214-3599
e-ISSN:2214-3602
Note:Publication type according to Uni Basel Research Database: Journal article
Language:English
Identification Number:
Last Modified:12 Apr 2019 13:19
Deposited On:03 Oct 2017 10:06

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