ADAM12 overexpression does not improve outcome in mice with laminin alpha2-deficient muscular dystrophy.
We have recently shown that overexpression of ADAM12 results in increased muscle regeneration and significantly reduced pathology in mdx, dystrophin deficient mice. In the present study, we tested the effect of overexpressing ADAM12 in dy(W) laminin-deficient mice. dy mice have a very severe clinical phenotype and would be expected to benefit greatly from enhanced regeneration. We found that dy(W) mice overexpressing ADAM12 indeed have increased muscle regeneration, as evidenced by increased numbers of muscle fibers expressing fetal myosin. However, overexpression of ADAM12 had no significant effect on overall health, as evidenced by body weight, and did not improve muscle pathology.
Guo
Ling T
LT
The Burnham Institute, 10901 North Torrey Pines Road, La Jolla, CA 92037, USA.
Shelton
G Diane
GD
Wewer
Ulla M
UM
Engvall
Eva
E
D051722:ADAM Proteins / Q000235:genetics / Q000378:metabolism*; D000072199:ADAM12 Protein; D000367:Age Factors; D000818:Animals; D015153:Blotting, Western / Q000379:methods; D001835:Body Weight / Q000502:physiology; D004195:Disease Models, Animal; D005786:Gene Expression Regulation / Q000502:physiology*; D007150:Immunohistochemistry / Q000379:methods; D007797:Laminin / Q000172:deficiency*; D051379:Mice; D018345:Mice, Knockout; D009137:Muscular Dystrophy, Animal / Q000235:genetics / Q000378:metabolism*; D012038:Regeneration / Q000235:genetics / Q000502:physiology
D007797:Laminin; C102645:laminin alpha 2; D051722:ADAM Proteins; D000072199:ADAM12 Protein; C096115:Adam12 protein, mouse