Author/Authors :
Skvortsova, V.L Pirogov Russian National Research Medical University, Moscow, Russia , Bachurin, S.O Institute of Physiologically Active Compounds of the Russian Academy of Sciences, Chernogolovka, Russia , Ustyugov, A.A Institute of Physiologically Active Compounds of the Russian Academy of Sciences, Chernogolovka, Russia , Kukharsky, M.S Pirogov Russian National Research Medical University, Moscow, Russia , Deikin, A.V Institute of Gene Biology of the Russian Academy of Sciences, Moscow, Russia , Buchman, V.L Institute of Physiologically Active Compounds of the Russian Academy of Sciences, Chernogolovka, Russia , Ninkina, N.N Institute of Physiologically Active Compounds of the Russian Academy of Sciences, Chernogolovka, Russia
Abstract :
Uncontrolled protein aggregation, accompanied by the formation of specific inclusions, is a major component of the pathogenesis of many common neurodegenerative diseases known as proteinopathies. The intermediate products of this aggregation are toxic to neurons and may be lethal. The development strategy of pathogenic therapy for proteinopathy is based on the design of drugs capable of both inhibiting proteinopathy progression and increasing the survival of affected neurons. The results of a decade-long research effort at leading Russian and international laboratories have demonstrated that Dimebon (Latrepirdine), as well as a number of its derivatives from a gamma-carboline group, show a strong neuroprotective effect and can mod-ulate the course of a neurodegenerative process in both in vitro and in vivo model systems. The accumulated data indicate that gamma-carbolines are promising compounds for the development of pathogenic therapy for proteinopathies.
Keywords :
transgenic animals , proteinopathy , gamma-carbolines , ALS, Dimebon
