Группа исследователей из Колумбийского университета под руководством Сержа Пржедборски (Serge Przedborski) создала полностью человеческую in vitro культуральную модель бокового амиотрофического склероза (БАС), с помощью которой был показан механизм, вызывающий гибель нейронов в случаях спорадического и семейного БАС.

В текущий момент БАС является неизлечимым заболеванием, ведущим к тяжелой инвалидизации и гибели большинства пациентов в течении 3-5 лет после постановки диагноза. Эффективных средств этитропной терапии на данный момент не создано, так как патологический механизм заболевания не понят до конца. Известно, что 90% случаев БАС - спорадические, 10% случаев являются семейными. Для семейных случаев установлена связь заболеваемости с мутациями примерно в 15 генах. Основную роль при этом приписывают мутация в гене супероксид-дисмутазы SOD1. До недавнего времени понять причины спорадических случаев БАС не удавалось. Перенос данных, полученных в мышиной модели БАС, на человека, был в основном неудачен, по всей видимости, из-за узости используемой модели (основана на нарушении функции гена SOD1).

И вот теперь работы в области человеческих моделей сдвинулись с мертвой точки - создана как сама модель, так и показан возможный универсальный механизм гибели нейронов.

Участниками группы были выделены астроциты головного и спинного мозга пациентов, погибших от БАС, после чего было произведено сокультивирование данных астроцитов с моторными нейронами человека, полученными из стволовых клеток. В итоге показано, что нейроны, которые росли в сокультуре с астроцитами больных БАС, гибли в течении двух недель путем некроптоза (програмируемый некроз). Гибель нейронов вызывали астроциты полученные от пациентов как с семейным, так и со спорадическим БАС. Нейроны, росшие с астроцитами от пациентов, погибших от других, не связанных с БАС причин, оставались жизнеспособны. Таким образом, впервые показан общий механизм нейронального поражения при всех формах БАС. Исследование пути астроцит-зависимого некроптоза мотонейронов сулит надежду разработки средств, замедляющих или предотвращающих развитие БАС.

Статья: Re DB, Le Verche V, Yu C, Amoroso MW, Politi KA, Phani S, Ikiz B, Hoffmann L, Koolen M, Nagata T, Papadimitriou D, Nagy P, Mitsumoto H, Kariya S, Wichterle H, Henderson CE, Przedborski S. Necroptosis Drives Motor Neuron Death in Models of Both Sporadic and Familial ALS. Neuron. 2014 Feb 4.

На гонорар не претендую, но новость переводная, с авторским текстом

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