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Connexins in skeletal muscle development and disease
Peter A Merrifield1, Dale W Laird1
1Department Anatomy and Cell Biology, Schulich School of Medicine and Dentistry, University of Western Ontario, London, ON, Canada N6A 5C1.
Seminars in Cell & Developmental Biology
|December 22, 2015
Summary
Connexins, proteins forming gap junctions, are crucial for muscle development and repair. Mutations in Cx43 cause oculodentodigital dysplasia (ODDD), potentially leading to skeletal muscle defects and weakness.
Area of Science:
- Cell Biology
- Developmental Biology
- Muscle Physiology
Background:
- Gap junctions, formed by connexins, facilitate intercellular communication.
- Connexins are present in developing and injured skeletal muscle, but absent in healthy adult muscle.
- Cx43 mutations are linked to oculodentodigital dysplasia (ODDD), a developmental disorder.
Purpose of the Study:
- To review the role of connexins in skeletal muscle development and repair.
- To discuss the impact of Cx43 mutations on muscle function and ODDD.
- To explore the potential myogenic origin of muscle weakness in ODDD patients.
Main Methods:
- Review of existing literature on connexins, skeletal muscle, and ODDD.
- Analysis of studies using gap junction blockers and cell culture models.
- Examination of genetically-modified mouse models with altered Cx43 function.
Main Results:
- Connexins are essential for myotube formation and muscle regeneration.
- Cx43 plays a significant role in skeletal muscle development.
- ODDD patients with Cx43 mutations may exhibit skeletal muscle abnormalities.
Conclusions:
- Connexins are vital for skeletal muscle formation and repair processes.
- Cx43 dysfunction due to ODDD mutations can impair muscle development and function.
- Further research is needed to clarify the myogenic contribution to ODDD-related muscle weakness.
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