Tg(ACTA1-PRKAG1*R70Q)3Lwit

The 3.6 kbp transgene contains a human skeletal muscle alpha-actin promoter (~2.2 kbp of the 5prime region of ACTA1), a full-length human AMP-activated protein kinase non-catalytic gamma1 (PRKAG1) cDNA sequence harboring the R70Q amino acid substitution tagged to the 5' end with a hemagglutinin (HA) epitope, all followed by the SV40 sequence. Immunoblot detects transgene expression in gastrocnemius, tibialis anterior, quadriceps and extensor digitorum longus muscles. There is little detectable expression in the soleus and low level expression in heart. Founder line 3 has approximately threefold increase in total gamma1 gene copy number compared with wildtype mice