Генетические причины

Есть семьи, в которых частота появления болезни Альцгеймера необычайно высока; этот факт уже сам по себе дает веские основания полагать, что по крайней мере некоторые формы заболевания связаны с наследственным фактором. «Виноват» ли некий дефектный ген (или гены)? Может ли элемент генетического аппарата сделать его обладателя особенно восприимчивым к

какому-либо фактору окружающей среды?

Типичные генетические болезни — это результат врожденных нарушений обмена; из-за аномалий в генетическом материале, ДНК, нарушается способность клеток производить определенный белок. Если этот белок — фермент, его можно идентифицировать по тому, что субстрат, превращение которого он обычно катализирует, содержится в организме в избытке (когда нет фермента, не идет и ускоряемая им реакция). Соответственно должно сократиться количество нормального продукта реакции. Иногда генетическую болезнь можно обнаружить и по хромосомной аномалии, как в случае синдрома Дауна.

Если в организме недостает какого- нибудь важного фермента, нарушение обмена веществ и сопутствующие ему патологические изменения наблюдаются обычно уже с рождения. Правда, некоторые болезни, генетическое происхождение которых доказано, «молчат» в первые десятилетия жизни. Хорея Геттингтона, для которой характерны двигательные расстройства, прогрессирующее слабоумие и гибель определенных групп нервных клеток мозга, проявляется не раньше, чем в сорокалетием возрасте. Как же может таиться так много лет генетическое нарушение, которое по определению имеется уже с момента зачатия?

Возможно, с возрастом повреждается далее уже исходно, по генетическим причинам нарушенная физиологическая система, например иммунная система; возможно, при старении усугубляется существовавшая изначально недостаточность какого-то важного белка, например фактора роста холинэргонических нейронов. В любом случае у некоторых членов семей, в которых высока частота заболевания, в мозгу должно не хватать этого белка, причем дефицит должен расти с возрастом.

Были обнаружены семьи, в которых по 10 и более человек — представителей 4—5 поколений, страдали слабоумием типа болезни Альцгеймера. В этих случаях признак, контролируемый дефектным геном, по-видимому, передается как аутосомно-доминантный, поскольку он проявляется у обоих полов и для полного эффекта достаточно, чтобы его нес лишь один из родителей. Генетический фактор может быть выявлен также с помощью анализа генеалогического древа — даже для тех семей, в которых болезнь обнаруживается только спорадически.

 

 

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