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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.

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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