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Scientists at MarSU and ITEB RAS have revealed the peculiarities of the functioning of skeletal muscle mitochondria in Duchenne dystrophy

20.01.2020 16:16:00

Scientists at MarSU and ITEB RAS have revealed the peculiarities of the functioning of skeletal muscle mitochondria in Duchenne dystrophy

Neuromuscular diseases, in particular, caused by genetic defects of myopathy, are the most frequent and often fatal hereditary diseases of a person. One of these pathologies is Duchenne's progressive muscular dystrophy.

Scientists at MarSU and ITEB RAS have revealed the peculiarities of the functioning of skeletal muscle mitochondria in Duchenne dystrophy
Scientists at MarSU and ITEB RAS have revealed the peculiarities of the functioning of skeletal muscle mitochondria in Duchenne dystrophy

Scientists at Mari State University, together with colleagues from the Institute of Theoretical and Experimental Biophysics (ITEB) of the Russian Academy of Sciences, with the support of the Russian Science Foundation (project No. 18-75-00011) studied the processes associated with dysregulation of calcium in the mitochondria of skeletal muscles of model mice suffering from this disease. The results of the study are published in the journal Biochimica et Biophysica Acta - Molecular Basis of Disease.

It turned out that with the development of Duchenne muscular dystrophy in the mitochondria of skeletal muscles, deep structural and functional rearrangements of the systems responsible for the transport of calcium ions occur. Such rearrangements lead to a decrease in the efficiency of absorption and deposition of calcium ions by organelles. Moreover, the mitochondria of such animals also become less resistant to the induction of calcium-dependent pores, which negatively affects their functional activity and increases the likelihood of triggering cell death processes.

The first author of the article, associate professor of the Department of Biochemistry, Cell Biology and Microbiology of the INP, Ph.D in Biology, Mikhail Vasilievich Dubinin summarizes: “The detected changes in the functioning of calcium-transporting systems of skeletal muscle mitochondria during Duchenne dystrophy appear to contribute to an increase in the level of free intracellular calcium in muscle tissue, which greatly accelerates the development of this disease. " Scientists believe that calcium-transporting mitochondrial systems can be used as targets, affecting which it is possible to correct the course of this neuromuscular disease. This will allow us to develop new approaches to the treatment of this serious illness.


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