Journal article
Sarcospan-Deficient Mice Maintain Normal Muscle Function
Molecular and cellular biology, Vol.20(5), pp.1669-1677
03/2000
DOI: 10.1128/MCB.20.5.1669-1677.2000
PMCID: PMC85350
PMID: 10669744
Abstract
Sarcospan is an integral membrane component of the dystrophin-glycoprotein complex (DGC) found at the sarcolemma of striated and smooth muscle. The DGC plays important roles in muscle function and viability as evidenced by defects in components of the DGC, which cause muscular dystrophy. Sarcospan is unique among the components of the complex in that it contains four transmembrane domains with intracellular N- and C-terminal domains and is a member of the tetraspan superfamily of proteins. Sarcospan is tightly linked to the sarcoglycans, and together these proteins form a subcomplex within the DGC. Stable expression of sarcospan at the sarcolemma is dependent upon expression of the sarcoglycans. Here we describe the generation and analysis of mice carrying a null mutation in the Sspngene. Surprisingly, the Sspn-deficient muscle maintains expression of other components of the DGC at the sarcolemma, and no gross histological abnormalities of muscle from the mice are observed. The Sspn-deficient muscle maintains sarcolemmal integrity as determined by serum creatine kinase and Evans blue uptake assays, and the Sspn-deficient muscle maintains normal force and power generation capabilities. These data suggest either that sarcospan is not required for normal DGC function or that the Sspn-deficient muscle is compensating for the absence of sarcospan, perhaps by utilizing another protein to carry out its function.
Details
- Title: Subtitle
- Sarcospan-Deficient Mice Maintain Normal Muscle Function
- Creators
- Connie S Lebakken - Departments of Physiology and Biophysics and Neurology, Howard Hughes Medical InstituteDavid P Venzke - Departments of Physiology and Biophysics and Neurology, Howard Hughes Medical InstituteRonald F Hrstka - Departments of Physiology and Biophysics and Neurology, Howard Hughes Medical InstituteChristina M Consolino - Departments of Physiology and Biophysics and Neurology, Howard Hughes Medical InstituteJohn A Faulkner - Departments of Physiology and Biophysics and Neurology, Howard Hughes Medical InstituteRoger A Williamson - Departments of Physiology and Biophysics and Neurology, Howard Hughes Medical InstituteKevin P Campbell - Departments of Physiology and Biophysics and Neurology, Howard Hughes Medical Institute
- Resource Type
- Journal article
- Publication Details
- Molecular and cellular biology, Vol.20(5), pp.1669-1677
- Publisher
- American Society for Microbiology
- DOI
- 10.1128/MCB.20.5.1669-1677.2000
- PMID
- 10669744
- PMCID
- PMC85350
- ISSN
- 0270-7306
- eISSN
- 1098-5549
- Language
- English
- Date published
- 03/2000
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Iowa Neuroscience Institute; Obstetrics and Gynecology
- Record Identifier
- 9984068374302771
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