Клубок фибрилл

На электронных микрофотографиях видно, что большинство составляющих клубок фибрилл состоят из двух волокон, спирально закрученных вокруг друг друга; их называют парными спиральными филаментами (ПСФ). Нормальные нейроны содержат три вида фибриллярных белковых образований, выполняющих в клетке структурную и транспортную функции: микротрубочки, микрофиламенты и нейрофиламенты (последние называют также промежуточными филаментами). Работы Д. Селко из Медицинской школы Гарвардского университета и У. Шлэпфера из Медицинской школы Пенсильванского университета позволили предположить, что ПСФ — это измененные нейрофиламенты. Аминокислотный состав очищенных нейрофиламентов во многих отношениях похож на таковой ПСФ, а моноклональные антитела, связывающие белки нейрофиламентов, реагируют и с некоторыми белками ПСФ; отсутствие полного сходства, возможно, объясняется тем, что препараты ПСФ очищены только частично.

Термин «амилоид», что значит «подобный крахмалу», употребляется для обозначения патологических скоплений в тканях богатых белком масс, примечательных главным образом своей способностью окрашиваться: если амилоид обработать красителем красный и наблюдать в поляризованном свете, видно зеленовато- желтое свечение. В некотором количестве амилоид имеется в мозгу у большинства старых людей, а у тех людей, которые страдают определенными хроническими заболеваниями, он содержится также в других органах (в частности, в печени и почках). Но обилие амилоида в мозговой ткани — диагностический признак болезни Альцгеймера, при которой, как я уже говорил, он обнаруживается в двух формах — как отложения внутри и снаружи кровеносных сосудов и как компонент сенильных бляшек. (Аномальные белки нейрофибриллярных клубков также могут окрашиваться, как амилоид.) Природа амилоидного белка варьирует; возможно, она зависит от того, какие белки-предшественники используются для его образования при том или ином патологическом состоянии.

Амилоид в кровеносных сосудах головного мозга — наиболее типичный признак болезни Альцгеймера.

 

 

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