ymjЯкутский медицинский журналYakut Medical Journal1813-19052312-1017ЯНЦ КМП10.25789/YMJ.2022.80.25ymj-314Research ArticleНАУЧНЫЕ ОБЗОРЫ И ЛЕКЦИИSCIENTIFIC REVIEWS AND LECTURESРоль ассортативных браков по глухоте в распространенности наследственной потери слухаThe role of assorted deafness marriages in the prevalence of hereditary hearing lossРомановГ. П.RomanovG. P.

Романов Георгий Прокопьевич – м.н.с.

Romanov Georgy Prokopievich – researcher

gpromanov@gmail.comБарашковН. А.BarashkovN. A.

Барашков Николай Алексеевич – к.б.н., в.н.с. , руковод. лаб.

Barashkov Nikolay Alekseevich – PhD in Biology, external researcher, head of the lab.

Якутский научный центр комплексных медицинских проблемРоссияYakut Science Centre of Complex Medical ProblemsRussian Federation2022070220250496100Copyright © Романов Г.П., Барашков Н.А., 20252025Романов Г.П., Барашков Н.А.Romanov G.P., Barashkov N.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://ymj.elpub.ru/jour/article/view/314

В статье представлен обзор, посвященный роли ассортативных браков по глухоте в распространении наследственной потери слуха. В 1883 г. Александр Грэйам Белл, знаменитый изобретатель телефона, впервые высказал предположение, что частые браки между глухими людьми могут привести к росту встречаемости глухоты, однако эта гипотеза не получила признания у его современников. В 2000-х гг. с развитием молекулярно-генетических исследований, позволивших идентифицировать одну из наиболее распространенных форм потери слуха, обусловленной мутациями гена GJB2, кодирующего белок межщелевых контактов – коннексин 26, эта гипотеза вновь была переосмыслена Уолтером Нэнсом. В серии работ ему и его соавторам удалось привести свидетельства в пользу того, что репродуктивные возможности глухих увеличились, и браки между глухими происходят не случайно, а по принципу ассортативности, что в сумме могло привести к увеличению распространенности 

The article presents an overview of the role of assorted deafness marriages in the spread of hereditary hearing loss. In 1883, Alexander Graham Bell, the famous inventor of the telephone, first suggested that frequent marriages between deaf people could lead to an increase in the incidence of deafness, but this hypothesis was not recognized by his contemporaries. In the 2000s, with the development of molecular genetic studies, which made it possible to identify one of the most common forms of hearing loss due to gene mutations GJB2, connexin 26, which encodes the interstitial contact protein, has been reinterpreted by Walter Nance. In the series of studies, he and his coauthors were able to show that the reproductive capabilities of the deaf have increased, and marriages between the deaf occur not by chance, but by the principle of assortativity, which in total could lead to an increase in the prevalence of one of the most frequent "connexin" forms of hearing loss.

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The authors declare that there are no conflicts of interest present.