Animal Models

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List of animal models (download .xls file)

Dystroglycanopathy models (download .doc file)

Dy-3k mice Ruegg  (download .doc file)

Dy-w mice Miller  (download .doc file)

Zebrafish models  (download .doc file)

Drosophila mutant  (download .doc file)

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About the LAMA2dy-w Mouse Model

Cure CMD together with TREAT-NMD and several leading scientists are working to establish standard operating protocols (SOPs) to standardize the care and evaluation of CMD animal models, starting with the laminin alpha 2 deficient mouse model, LAMA2dy-w.   This effort led by Dr. Raffaella Willmann focused on a model of Laminin Alpha 2 Related CMD (Merosin Deficient, MDC1A) and began by selecting readout parameters in mice that are appropriate to show the efficacy of a new potential drug and are related to patient outcomes. Then, experienced scientists pooled their know-how to produce effective and reproducible step-by-step protocols that are now available to researchers on this page. The availability and use of common, standard protocols will lead to focused evaluations, allowing comparative efficacy across CMD translational research using the same animal model and SOPs and eventually lead to an accelerated development of promising potential treatments.

As part of this effort, the LAMA2dy-w mouse was deposited in April 2011 at Jackson laboratories and is now on a fully congenic on a C57BL/6J background by marker assisted backcrossing at 5 generations, with a live colony maintained at Jackson laboratories. Cure CMD encourages all proof of concept and preclinical trials in this animal model to be done with mice obtained from Jackson laboratories to remove any bias of academic based colony genetic drift and background variability.

To order the LAMA2dy-w, click here to visit the JAX website

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Thanks goes to the following people who contributed or are contributing to the availability of standard protocols:

Dr. Valerie Allamand, INSERM, France
Dr. Dean Burkin, University of Nevada
Dr. Anne Connolly, Washington School of Medicine
Dr. Janice Dominov, Boston Biomedical Research Institute
Dr. Madeleine Durbeej-Hjalt, Lund University, Sweden
Dr. Eva Engvall, Sanford-Burnham Medical Research Institute
Dr. Mahasweta Girgenrath, Boston University
Dr. Jeff Miller, Boston Biomedical Research Institute
Dr. Kanneboyina Nagaraju, CNMC, Washington
Dr. Markus Rüegg, University of Basel, Switzerland
Dr. Volker Straub, University of Newcastle, UK
Dr. Rich Lovering, University of Maryland School of Medicine
Dr. Gregorio Valdez, Virginia Tech Carilion Research Institute
Dr. Jean-Yves Hogrel, Institut de Myologie, France