Date: 11 months ago   Category: Studies

Scientists from Russia have found out how there are rare forms of muscle weakness - RIA Novosti, 8/22/2018

MOSCOW, 22 Aug – RIA Novosti. Molecular biologists from RAS have found out why breakages in a gene of TPM2 do muscles of the person weak and prevent him to breathe and operate vocal chords and a throat normally. Their conclusions have been presented in the Biochemical and Biophysical Research Communications.© magazine of the Photo: Scientific Mikal Schlosser: gene therapy has stopped dystrophy of muscles at a mouse" information Obtained by us will help us to understand how muscles normal are reduced and at development of myopathies and also will help with search of targets for therapeutic influence at early stages of display of a disease. Subsequently they can be used for testing of medicines", – Yury Borovikov from Institute of cytology of RAS in Moscow whose words are transferred by the press service of the Russian scientific fund tells. At once several million people living today on Earth suffer from a myopathy – violations in work of muscles. Besides various forms of dystrophy connected with destruction of muscular cages or serious problems in their activity there are also other forms of this disease arising in rather healthy muscle fibers. The striking example of it is a so-called not crimson myopathy. At her development in muscles there are strange threadlike and diamond-shaped structures, so-called not crimson bodies preventing their work and causing weakness in all muscles of the person. In the most hard cases her carriers almost can't independently breathe and move with great difficulty. Scientists, as Borovikov notes, know long ago that this form of muscle weakness arises because of a single mutation in a gene and protein TPM2. They play a role of a peculiar "sensor of teams" in cells of muscles – TPM2 constantly watches concentration of ions of calcium in a cage and "includes" other squirrels who are responsible for reduction of a cage if their share reaches there is nobody a threshold. © Scientists from Russia have found Fotolia/Kzenon in muscles mysterious proteinaceous "balls" the Russian biologists have found out how mutations in this gene influence his work and "communication" with other enzymes, having grown up the culture of cells of the muscles of a rabbit containing the defective version of TPM2 and its normal versions marked by means of special glowing proteins. Processing these cages calcium ions, scientists forced them to be laid off and watched how the luminescence of the molecules connected with TPM2 changed. It has allowed them to understand to what other parts of a cage this enzyme in the normal and mutated state connects. As have shown these experiences, emergence even of one "typo" in TPM2 sharply reduced sensitivity of protein to external signals – for "inclusion" of muscles it was required almost 10 times more calcium ions, than in normal cages. What the mutant version of protein has begun "to get jammed" was the cause of it. She extremely reluctantly switched from one state in another, interacting with one more enzyme, troponiny. He operates the provision of the molecules TPM2 in muscle fibers, helping him "to conduct" muscle work. © Photo: Roskosmos/Yury Malenchenko to Pass into Bee's image bank



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