Палочки- агрегаты прионов

 П. Мерц и Р. Саммер- вил из Нью-Йоркского государственного института фундаментальных исследований патологии развития обнаружили, что высокоочишенные препараты фактора, вызывающего скрэпи, содержат многочисленные палочковидные структуры, и предположили, что они-то и есть инфекционный агент. Прузинер считает палочки агрегатами прионов. Если палочки окрасить конго красным, они становятся очень похожи на амилоид, присутствующий в мозгу у овец, больных скрэпи, и у некоторых людей, страдающих болезнью Крейцфельдта—Якоба. Как полагает Прузинер, этот амилоид представляет собой именно скопления прионовых палочек. По его мнению, амилоид, обнаруживаемый при болезни Альцгеймера, — тоже скопления прионов, которые и могут быть ее возбудителем.

Д. Гайдушек из Национального института неврологических расстройств и инсульта и его коллеги наблюдали аномальные структуры, построенные из закрученных волокон, в мозгу животных, больных скрэпи, и людей, страдающих болезнью Крейцфельд- та—Якоба или куру. (Куру — это неврологическое расстройство, распространенное в некоторых племенах Новой Гвинеи. За доказательство заразности куру и болезни Крейцфельдта—Якоба Гайдушек был удостоен в 1976 г. Нобелевской премии.) Они предположили, что эти волокнистые образования и являются инфекционным агентом. Подобные структуры отсутствуют при болезни Альцгеймера, однако некоторые их иммунологические свойства совершенно такие же, как у нейрофибриллярных клубков.

Если болезнь Альцгеймера вызывается инфекционным агентом, она должна быть заразной; однако попытки передать ее лабораторным животным были безуспешными. Возможно, в данном случае для того чтобы произошло заражение болезнетворным агентом (если таковой имеется), необходимо определенное генетическое «обеспечение», сопутствующее иммунологическое расстройство или предварительное воздействие какого-то токсина из внешней среды.

 

 

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