Increased protein degradation results from elevated free calcium levels found in muscle from mdx mice
- 1 October 1988
- journal article
- research article
- Published by Springer Science and Business Media LLC in Nature
- Vol. 335 (6192), 735-738
- https://doi.org/10.1038/335735a0
Abstract
The defective gene responsible for Duchenne muscular dystrophy in humans and the dystrophic condition in mdx mice results in a lack of dystrophin at first thought to be localized to the triads, but more recently found on the cytoplasmic side of the sarcolemma of skeletal muscle fibres. Because the total calcium content of dystrophic fibres is significantly raised, we have compared the intracellular free calcium concentration [( Ca2+]i) in skeletal muscle in normal and mdx mice. We find that [Ca2+]i is markedly elevated in mdx muscle fibres compared with normal fibres, both at rest and during stimulation. By measuring protein degradation rates and manipulating [Ca2+]i, we have been able to demonstrate directly that the elevation of [Ca2+]i in mdx fibres results in an enhanced net degradation of muscle proteins.Keywords
This publication has 20 references indexed in Scilit:
- Immunoelectron microscopic localization of dystrophin in myofibresNature, 1988
- The Duchenne muscular dystrophy gene product is localized in sarcolemma of human skeletal muscleNature, 1988
- Free cytoplasmic Ca ++ at rest and after cholinergic stimulus is increased in cultured muscle cells from Duchenne muscular dystrophy patientsNeurology, 1988
- Negative immunostaining of Duchenne muscular dystrophy(DMD) and mdx muscle surface membrane with antibody against synthetic peptide fragment predicted from DMD cDNA.Proceedings of the Japan Academy, Series B, 1988
- Dystrophin: The protein product of the duchenne muscular dystrophy locusCell, 1987
- Subcellular fractionation of dystrophin to the triads of skeletal muscleNature, 1987
- Measurements of calcium and other elements in muscle biopsy samples from patients with Duchenne Muscular DystrophyClinica Chimica Acta; International Journal of Clinical Chemistry, 1985
- Calcium and magnesium content in fetuses at risk and prenecrotic Duchenne muscular dystrophyNeurology, 1984
- Muscle calcium and magnesium content in Duchenne muscular dystrophyNeurology, 1982
- Role of intracellular calcium in promoting muscle damage: A strategy for controlling the dystrophic conditionCellular and Molecular Life Sciences, 1978