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CMD Preclinical research

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.

Laminin deficiency mouse

Mouse

Disease

Description
Lama2dy/dy MDC1A http://jaxmice.jax.org/strain/000641.html
Lama2dyW/dyW MDC1A http://jaxmice.jax.org/strain/013786.html
Lama2dy2J/dy2j MDC1A http://jaxmice.jax.org/strain/000524.html
Lama2dy-6J MDC1A http://jaxmice.jax.org/strain/003589.html
Lama2dy-7J MDC1A http://jaxmice.jax.org/strain/005635.html
Lama2dy3k/dy3k MDC1A http://www.ncbi.nlm.nih.gov/pubmed/9326364
Dystroglycan deficiency/modification mouse

Mouse

Disease

Description
Dag1-null http://jaxmice.jax.org/strain/006836.html
MCK-DG null http://www.ncbi.nlm.nih.gov/pubmed/12230980
GFAP-Cre/DG null http://www.ncbi.nlm.nih.gov/pubmed/12140559
MORE-DG null WWS-like http://www.ncbi.nlm.nih.gov/pubmed/18923033
P0-DG null http://www.ncbi.nlm.nih.gov/pubmed/12797959
DG chimera http://www.ncbi.nlm.nih.gov/pubmed/10610181
POMT1-/- WWS http://www.ncbi.nlm.nih.gov/pubmed/15383666
POMGnT1-/- MEB http://www.ncbi.nlm.nih.gov/pubmed/16458488
FKRPTyr307Asn MEB http://www.ncbi.nlm.nih.gov/pubmed/19155270
Fukutin Chimera FCMD http://www.ncbi.nlm.nih.gov/pubmed/12783852
Largemyd MDC1D http://jaxmice.jax.org/strain/000226.html
http://jaxmice.jax.org/strain/000300.html
http://jaxmice.jax.org/strain/005350.html
Largeenr MDC1D http://jaxmice.jax.org/strain/002491.html
Lamin deficiency mouse

Mouse

Disease

Description
Lamin A/C -/- EDMD http://jaxmice.jax.org/strain/009125.html
Lamin A/C H222P EDMD http://www.ncbi.nlm.nih.gov/pubmed/15548545
Lamin A/C N195K http://www.ncbi.nlm.nih.gov/pubmed/15972724
Lamin A/C M371K EDMD http://www.ncbi.nlm.nih.gov/pubmed/16825283
Integrin deficiency mouse

Mouse

Disease

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

Mouse

Disease

Description
Col6A1 -/- Bethlem http://www.ncbi.nlm.nih.gov/pubmed/9817932
Emerin KO EDMD1 http://www.ncbi.nlm.nih.gov/pubmed/16403804
Nesprin-1 ΔKASH EDMD-like http://www.ncbi.nlm.nih.gov/pubmed/19008300
LAP2 -/- http://www.ncbi.nlm.nih.gov/pubmed/20039368
Sil1 (Woozy mouse) Marinesco Sjogren syndrome http://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. James Dowling, Univ of Michigan
  • Dr. Markus Ruegg
  • Dr. Jeffrey Miller, BBRI
  • Dr. Anne Rutkowski

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