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CMD Preclinical research

Mouse models for CMD

In preparation for the first CMD Therapeutic Target Conference in July 2009 organized by Drs. Carsten Bonnemann and Anne Rutkowski (Cure CMD), an animal model working group pulled together preliminary data for one of the conference focus areas: CMD animal models. This list represents the existing CMD animal models as of July 2009 with several recently made animal models soon to be published. The 2 1/2 day conference funded by NINDS, NIAMS, ORD, Cure CMD, MDA, Debiopharm and PTC Therapeutics brought together an international group of scientists and clinicians to discuss CMD pathophysiology and targets.

Dystroglycan deficiency/modification mouse
Laminin deficiency mouse

Mouse
</strong

Disease

Description
Lama2dy/dyMDC1Ahttp://jaxmice.jax.org/strain/000641.html
Lama2dyW/dyWMDC1Ahttp://jaxmice.jax.org/strain/013786.html
Lama2dy2J/dy2jMDC1Ahttp://jaxmice.jax.org/strain/000524.html
Lama2dy-6JMDC1Ahttp://jaxmice.jax.org/strain/003589.html
Lama2dy-7JMDC1Ahttp://jaxmice.jax.org/strain/005635.html
Lama2dy3k/dy3kMDC1Ahttp://www.ncbi.nlm.nih.gov/pubmed/9326364

Mouse
</strong

Disease

Description
Dag1-nullhttp://jaxmice.jax.org/strain/006836.html
MCK-DG nullhttp://www.ncbi.nlm.nih.gov/pubmed/12230980
GFAP-Cre/DG nullhttp://www.ncbi.nlm.nih.gov/pubmed/12140559
MORE-DG nullWWS-likehttp://www.ncbi.nlm.nih.gov/pubmed/18923033
P0-DG nullhttp://www.ncbi.nlm.nih.gov/pubmed/12797959
DG chimerahttp://www.ncbi.nlm.nih.gov/pubmed/10610181
POMT1-/-WWShttp://www.ncbi.nlm.nih.gov/pubmed/15383666
POMGnT1-/-MEBhttp://www.ncbi.nlm.nih.gov/pubmed/16458488
FKRPTyr307AsnMEBhttp://www.ncbi.nlm.nih.gov/pubmed/19155270
Fukutin ChimeraFCMDhttp://www.ncbi.nlm.nih.gov/pubmed/12783852
LargemydMDC1Dhttp://jaxmice.jax.org/strain/000226.html
http://jaxmice.jax.org/strain/000300.html
http://jaxmice.jax.org/strain/005350.html
LargeenrMDC1Dhttp://jaxmice.jax.org/strain/002491.html
Lamin deficiency mouse

Mouse
</strong

Disease

Description
Lamin A/C -/-EDMDhttp://jaxmice.jax.org/strain/009125.html
Lamin A/C H222PEDMDhttp://www.ncbi.nlm.nih.gov/pubmed/15548545
Lamin A/C N195Khttp://www.ncbi.nlm.nih.gov/pubmed/15972724
Lamin A/C M371KEDMDhttp://www.ncbi.nlm.nih.gov/pubmed/16825283
Integrin deficiency mouse

Mouse
</strong

Disease

Description
Integrin α7 -/-http://www.ncbi.nlm.nih.gov/pubmed/16003770
Integrin α7 -/-/mdxhttp://www.ncbi.nlm.nih.gov/pubmed/16684813
Integrin b2 -/-LADIhttp://jaxmice.jax.org/strain/003329.html
Other deficiencies mouse

Mouse
</strong

Disease

Description
Col6A1 -/-Bethlemhttp://www.ncbi.nlm.nih.gov/pubmed/9817932
Emerin KOEDMD1http://www.ncbi.nlm.nih.gov/pubmed/16403804
Nesprin-1 ΔKASHEDMD-likehttp://www.ncbi.nlm.nih.gov/pubmed/19008300
LAP2 -/-http://www.ncbi.nlm.nih.gov/pubmed/20039368
Sil1 (Woozy mouse)Marinesco Sjogren syndromehttp://jaxmice.jax.org/strain/003777.html

Thanks goes to the following people who contributed to information on this page:

  • Dr. Yiumo Chan, Carolinas Medical Center
  • Dr. Sue Brown, Royal Vetinary College
  • Dr. Dan Michele, Univ of Michigan
  • Dr. Jim Dowling, Univ of Michigan
  • Dr. Markus Ruegg
  • Dr. Jeffrey Miller, BBRI
  • Dr. Anne Rutkowski

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