Resources for Scientists
Animal Models
​
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)
​
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
​
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
Standard Operating Protocols (SOPs)
MD_M.2.2.001
rev 25 Mar 2014
Serum Creatine Kinase analysis in mouse models of muscular dystrophy
MD_M.2.2.002
rev 08 Apr 2014
Behavioral Phenotyping for neonates: Righting reflex
Email: Christine DiDonato, Laurent Bogdani
CMD_M.2.2.001
rev 14 Oct 2013
Non-invasive measurement of ankle flexion/extension torque for longitudinal follow-up of strength in small rodents
Email: Jean-Yves Hogrel
MDC1A_M.1.2.001
rev 13 Jan 2014
Assessment of peripheral nerve pathology in laminin-alpha-2-deficient mice
Email: Jeffrey B. Miller
MDC1A_M.1.2.002
rev 28 Jan 2014
Quantitative determination of minimal Feret’s diameter, including the evaluation of the percentage of centralized nuclei, fiber numbers, cross-sectional area and the percentage of fibrosis
Email: Markus A. Rüegg
MDC1A_M.1.2.003
rev 28 Jan 2014
Histopathology in Masson Trichrome stained muscle sections
Email: Markus A. Rüegg
MDC1A_M.1.2.004
rev 28 Jan 2014
Histopathology in Hematoxylin & Eosin stained muscle sections
Email: Markus A. Rüegg
MDC1A_M.1.2.005
rev 25 Jan 2014
Inflammation quantification in the LAMA2dy-w mouse
Email: Dean Burkin
MDC1A_M.1.2.006
10 July 2013
Neuromuscular junction imaging on whole muscles and muscle sections in CMD animal modelsdy-w mouse
Email: Gregorio Valdez
MDC1A_M.2.1.001
rev 25 Jan 2014
Recommendations on the care and handling of the MDC1A mouse model LAMA2dy-w
Email: Janice Dominov
MDC1A_M.2.2.001
07 Dec 2013
Use of Grip Strength Meter to Assess the Limb Strength of dyw Mice
Email: Anne Connolly
MDC1A_M.2.2.002
05 Dec 2013
Single Muscle Contractile Measurements In Vivo and In Situ
Email: Rich Lovering
XLMTM_D.2.2.001
26 Mar 2014
Gait assessment in a canine model of XLMTM using an instrumented carpet
Email: Melissa A Goddard
XLMTM_D.2.2.002
26 Mar 2014
Respiratory assessment of a canine model of XLMTM using non-invasive respiratory impedence plethysmography
Email: Melissa A Goddard
Fibroblasts
Jan 2014
Primary Firbroblast Culture from Human Skin Biopsy
Email: Yaqun Zou
Myoblasts
Jan 2014
Primary Myoblast Culture from Fresh Human Muscle Biopsy
Email: Yaqun Zou
Lymphocytes
Aug 2004
EBV Immortalization of Lymphoblasts
Email: Marina Mora, Euro Bio Bank