Title |
Leaky ryanodine receptors in β-sarcoglycan deficient mice: a potential common defect in muscular dystrophy
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Published in |
Skeletal Muscle, May 2012
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DOI | 10.1186/2044-5040-2-9 |
Pubmed ID | |
Authors |
Daniel C Andersson, Albano C Meli, Steven Reiken, Matthew J Betzenhauser, Alisa Umanskaya, Takayuki Shiomi, Jeanine D’Armiento, Andrew R Marks |
Abstract |
Disruption of the sarcolemma-associated dystrophin-glycoprotein complex underlies multiple forms of muscular dystrophy, including Duchenne muscular dystrophy and sarcoglycanopathies. A hallmark of these disorders is muscle weakness. In a murine model of Duchenne muscular dystrophy, mdx mice, cysteine-nitrosylation of the calcium release channel/ryanodine receptor type 1 (RyR1) on the skeletal muscle sarcoplasmic reticulum causes depletion of the stabilizing subunit calstabin1 (FKBP12) from the RyR1 macromolecular complex. This results in a sarcoplasmic reticular calcium leak via defective RyR1 channels. This pathological intracellular calcium leak contributes to reduced calcium release and decreased muscle force production. It is unknown whether RyR1 dysfunction occurs also in other muscular dystrophies. |
X Demographics
Geographical breakdown
Country | Count | As % |
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United Kingdom | 1 | 100% |
Demographic breakdown
Type | Count | As % |
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Members of the public | 1 | 100% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
United States | 1 | 2% |
Germany | 1 | 2% |
Brazil | 1 | 2% |
Unknown | 55 | 95% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Student > Ph. D. Student | 16 | 28% |
Student > Master | 9 | 16% |
Researcher | 9 | 16% |
Student > Bachelor | 6 | 10% |
Professor | 5 | 9% |
Other | 4 | 7% |
Unknown | 9 | 16% |
Readers by discipline | Count | As % |
---|---|---|
Agricultural and Biological Sciences | 19 | 33% |
Biochemistry, Genetics and Molecular Biology | 13 | 22% |
Medicine and Dentistry | 7 | 12% |
Pharmacology, Toxicology and Pharmaceutical Science | 1 | 2% |
Nursing and Health Professions | 1 | 2% |
Other | 3 | 5% |
Unknown | 14 | 24% |