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Why the muscle swelling in infantile cerebral palsy occur

Doctor Nikonov N.B. is the first doctor who drew attention to the swelling of muscle cells and studied it. For further research, he needed assistance from the institute in the city of Osaka. After a careful study of the issue, a team of specialists led by Professor Kiyotoshi Sekiguchi responded to Nikonov N.B.

The lymphatic system, consisting of vessels and lymph nodes, regulates the total number of cell waste in muscle tissues. When faults occur in the cleaning mechanism, toxins begin to accumulate in the muscle tissues. This leads to a condition called swelling.

The presence of swelling in the muscles of a child leads to their immobility in infantile cerebral palsy.

• Swelling can be fatal, for example, if breathing stops due to pulmonary edema.

• Muscle swelling in the embryo causes premature birth and the appearance of premature infants.

Until 2017, the molecular mechanisms being the basis of the pathological development of the lymphatic system in the late stages of embryonic development, in which the underdeveloped lymphatic system is transformed into mature lymphatic vessels, were unknown. For this reason, I offered to cooperate with a well-known scientist from the University of Osaka. The main task of Kiyotoshi Sekiguchi was the determination of protein which is the main building element for lymphatic vessels.

Polydome is a large-sized protein, which is a building material for the lymphatic vessel walls and simultaneously interacting with the α9β1 integrin receptors.

The main reason that the underdeveloped lymphatic system of the embryo ceases to grow and develop is the fact that the building protein ceases to be synthesized by endothelial cells and mesenchymal cells. As a consequence, the waste is collected in the cells and the muscle swelling occurs, which does not allow the muscular tissues to function normally. It is this mechanism of the emergence of muscles immobility in infantile cerebral palsy.

Based on his research in the field of genetic engineering, Professor Kiyotoshi Sekiguchi determined that in case of a shortage of polydome protein, the lymphatic system is unable to develop at the same rate as the muscle tissue. The scientist analyzed the location of the protein in embryos and concluded that it is this element which is responsible for constructing the lymphatic system of vessels during the entire embryonic development.

“Polydome is secreted by mesenchymal cells. It is an integral part of the process of creating the lymphatic system vessels in the late stages of embryo development”.

The lymphatic bed is the second vascular system used by the human body to perform immune functions and eliminate the metabolic byproducts of muscle cells. Insufficient development of the hierarchy of vessels with a densely branched capillary network is crucial for the development of muscle swelling and as a consequence of the development of infantile cerebral palsy of varying severity. The main intercellular matrix protein intended to create the lymphatic system is a polydome (also called Svep1).

The polydome protein deficiency indicates an obvious swelling of the muscles that participate in breathing: in all newborns immediately after birth, breathing stops due to respiratory failure. In the absence of polydome protein, the lymphatic vessels are not constructed. The embryo, having primitive lymphatic plexuses, is unable to grow new capillaries and form lymphatic vessels collecting the lymph.

Conclusions: Polydome builds new lymphatic vessels in embryonic development.

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