1. Lmo7 is dispensable for skeletal muscle and cardiac function
- Author
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Kirk L. Peterson, Mary C. Esparza, Jianlin Zhang, Kirk U. Knowlton, Dieu Hung Lao, Nancy D. Dalton, William H. Bradford, Ju Chen, Richard L. Lieber, Shannon N. Bremner, Indroneal Banerjee, Jennifer Veevers, and Yusu Gu
- Subjects
musculoskeletal diseases ,Cardiac function curve ,medicine.medical_specialty ,MAP Kinase Signaling System ,Physiology ,Gene Expression ,Biology ,Mice ,Degenerative disease ,Internal medicine ,medicine ,Animals ,X-linked muscular dystrophy ,Muscular dystrophy ,Muscle, Skeletal ,Transcription factor ,Mice, Knockout ,Myocardium ,Cardiac muscle ,Skeletal muscle ,Heart ,Articles ,Cell Biology ,LIM Domain Proteins ,medicine.disease ,Muscular Dystrophy, Emery-Dreifuss ,Mice, Inbred C57BL ,medicine.anatomical_structure ,Endocrinology ,ITGA7 ,Transcription Factors - Abstract
Emery-Dreifuss muscular dystrophy (EDMD) is a degenerative disease primarily affecting skeletal muscles in early childhood as well as cardiac muscle at later stages. EDMD is caused by a number of mutations in genes encoding proteins associated with the nuclear envelope (e.g., Emerin, Lamin A/C, and Nesprin). Recently, a novel protein, Lim-domain only 7 ( lmo7) has been reported to play a role in the molecular pathogenesis of EDMD. Prior in vitro and in vivo studies suggested the intriguing possibility that Lmo7 plays a role in skeletal or cardiac muscle pathophysiology. To further understand the in vivo role of Lmo7 in striated muscles, we generated a novel Lmo7-null ( lmo7−/−) mouse line. Using this mouse line, we examined skeletal and cardiac muscle physiology, as well as the role of Lmo7 in a model of muscular dystrophy and regeneration using the dystrophin-deficient mdx mouse model. Our results demonstrated that lmo7−/− mice had no abnormalities in skeletal muscle morphology, physiological function, or regeneration. Cardiac function was also unaffected. Moreover, we found that ablation of lmo7 in mdx mice had no effect on the observed myopathy and muscular regeneration exhibited by mdx mice. Molecular analyses also showed no changes in dystrophin complex factors, MAPK pathway components, and Emerin levels in lmo7 knockout mice. Taken together, we conclude that Lmo7 is dispensable for skeletal muscle and cardiac physiology and pathophysiology.
- Published
- 2015