Sarcolipin overexpression impairs myogenic differentiation in Duchenne muscular dystrophy

American Journal of Physiology. Cell Physiology
Nandita NiranjanGopal J Babu

Abstract

Reduction in the expression of sarcolipin (SLN), an inhibitor of sarco(endo)plasmic reticulum (SR) Ca2+-ATPase (SERCA), ameliorates severe muscular dystrophy in mice. However, the mechanism by which SLN inhibition improves muscle structure remains unclear. Here, we describe the previously unknown function of SLN in muscle differentiation in Duchenne muscular dystrophy (DMD). Overexpression of SLN in C2C12 resulted in decreased SERCA pump activity, reduced SR Ca2+ load, and increased intracellular Ca2+ ( Ca i 2+ ) concentration. In addition, SLN overexpression resulted in altered expression of myogenic markers and poor myogenic differentiation. In dystrophin-deficient dog myoblasts and myotubes, SLN expression was significantly high and associated with defective Ca i 2+ cycling. The dystrophic dog myotubes were less branched and associated with decreased autophagy and increased expression of mitochondrial fusion and fission proteins. Reduction in SLN expression restored these changes and enhanced dystrophic dog myoblast fusion during differentiation. In summary, our data suggest that SLN upregulation is an intrinsic secondary change in dystrophin-deficient myoblasts and could account for the Ca i 2+ m...Continue Reading

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Citations

Nov 21, 2020·American Journal of Physiology. Heart and Circulatory Physiology·Satvik MareeduGopal J Babu
Nov 19, 2020·Veterinary Sciences·Joseph M AutryStephanie J Valberg
Mar 19, 2021·Frontiers in Physiology·Naresh C BalMuthu Periasamy
Apr 27, 2021·Frontiers in Physiology·Satvik MareeduGopal J Babu
May 20, 2021·Scientific Reports·Jamie FranciscoDominic P Del Re

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Methods Mentioned

BETA
PCR
electrophoresis

Software Mentioned

Imaging Workbench
GraphPad Prism
Image Laboratory
Cell Sense standard
Mito Stress
Excel

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