Conclusions
These findings demonstrate that loss of Lmo7 in mice causes myopathic phenotypes similar to those seen in other EDMD mouse models.
Methods
The skeletal muscle and cardiac phenotypes were analyzed in a newly generated Lmo7-null mouse using histological analysis, echocardiography, and various neuromuscular tests to determine if Lmo7 was important for skeletal muscle and cardiac function.
Results
Lmo7-null mice had growth retardation, decreased fiber size, and impaired skeletal muscle and cardiac function. Lmo7-null mice also had lower levels of phosphorylated retinoblastoma (Rb), extracellular signal-regulated kinase, and c-Jun N-terminal kinase, which is consistent with altered Rb and mitogen-activated protein kinase signaling. Conclusions: These findings demonstrate that loss of Lmo7 in mice causes myopathic phenotypes similar to those seen in other EDMD mouse models.