Dubinin 2020 Biochim Biophys Acta Bioenerg

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Dubinin MV, Talanov EY, Tenkov KS, Starinets VS, Mikheeva IB, Belosludtsev KN (2020) Transport of Ca2+ and Ca2+-dependent permeability transition in heart mitochondria in the early stages of duchenne muscular dystrophy. Biochim Biophys Acta Bioenerg [Epub ahead of print].

» PMID: 32569663

Dubinin Mikhail, Talanov Eugeny Yu, Tenkov Kirill S, Starinets Vlada S, Mikheeva Irina B, Belosludtsev Konstantin (2020) Biochim Biophys Acta Bioenerg

Abstract: Duchenne muscular dystrophy (DMD) is a progressive skeletal muscle disease that is associated with severe cardiac complications in the late stages. Significant mitochondrial dysfunction is reportedly responsible for the development of cardiomyopathy with age. At the same time, adaptive changes in mitochondrial metabolism in cardiomyocytes were identified in the early stages of DMD. In this work, we evaluate the functioning of calcium transport systems (MCU and NCLX), and MPT pore in the heart mitochondria of young dystrophin-deficient mice. As compared to wild-type animals, heart mitochondria of mdx mice have been found to be more efficient both in respect to Ca2+ uniport and Na+-dependent Ca2+ efflux. The data obtained indicate that the increased rate of Ca2+ uptake by heart mitochondria of mdx mice may be due to an increase in the ratio of MCU and MCUb subunits. In turn, an increase in the rate of Ca2+ efflux from organelles in DMD may be the result of a significant increase in the level of NCLX. Moreover, the heart mitochondria of mdx mice were more resistant to MPT pore opening, which may be due to an increase in the microviscosity of mitochondrial membranes of DMD mice. At the same time, the level of putative MPT pore proteins did not change. The paper discusses the effect of rearrangements of the mitochondrial proteome involved in the transport and accumulation of calcium on the adaptation of this organ to DMD.

Keywords: Ca(2+) uniporter, Duchenne muscular dystrophy, Heart, Mitochondria, Mitochondrial permeability transition, NCLX Bioblast editor: Plangger M

Labels: MiParea: Respiration  Pathology: Myopathy  Stress:Permeability transition  Organism: Mouse  Tissue;cell: Heart  Preparation: Isolated mitochondria 

Regulation: Ion;substrate transport  Coupling state: LEAK, OXPHOS  Pathway:HRR: Oxygraph-2k