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

Download: .pdf file

Email: Kanneboyina Nagaraju

MD_M.2.2.002

rev 08 Apr 2014

Behavioral Phenotyping for neonates: Righting reflex

Download .pdf file

Email: Christine DiDonatoLaurent 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

Download .pdf file

Email: Jean-Yves Hogrel

MDC1A_M.1.2.001

rev 13 Jan 2014

Assessment of peripheral nerve pathology in laminin-alpha-2-deficient mice

Download .pdf file

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

Download .pdf file

Email: Markus A. Rüegg

MDC1A_M.1.2.003

rev 28 Jan 2014

Histopathology in Masson Trichrome stained muscle sections

Download .pdf file

Email: Markus A. Rüegg

MDC1A_M.1.2.004

rev 28 Jan 2014

Histopathology in Hematoxylin & Eosin stained muscle sections

Download .pdf file

Email: Markus A. Rüegg

MDC1A_M.1.2.005

rev 25 Jan 2014

Inflammation quantification in the LAMA2dy-w mouse

Download .pdf file

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

Download .pdf file

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

Download .pdf file

Email: Janice Dominov

MDC1A_M.2.2.001

07 Dec 2013

Use of Grip Strength Meter to Assess the Limb Strength of dyw Mice

Download .pdf file

Email: Anne Connolly

MDC1A_M.2.2.002

05 Dec 2013

Single Muscle Contractile Measurements In Vivo and In Situ

Download .pdf file

Email: Rich Lovering

XLMTM_D.2.2.001

26 Mar 2014

Gait assessment in a canine model of XLMTM using an instrumented carpet

Download .pdf file

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

Download .pdf file

Email: Melissa A Goddard

Fibroblasts

Jan 2014

Primary Firbroblast Culture from Human Skin Biopsy

Download .pdf file

Email: Yaqun Zou

Myoblasts

Jan 2014

Primary Myoblast Culture from Fresh Human Muscle Biopsy

Download .pdf file

Email: Yaqun Zou

Lymphocytes

Aug 2004

EBV Immortalization of Lymphoblasts

Download .doc file

Email: Marina Mora, Euro Bio Bank

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