Vol 42(2008) N 5 p. 747-761;
N.N. Ninkina1,2, A.A. Ustyugov2, V.L. Buchman1
Modeling synucleinopathies in genetically modified animals: Successes and failures1School of Biosciences, Cardiff University, Museum Avenue, Cardiff, CF10 3US, UK
2Institute of Physiologically Active Compounds, Russian Academy of Sciences, Chernogolovka, Moscow Region, 142432, Russia
Received - 2008-04-28; Accepted - 2008-04-28
The synuclein family and particularly α-synuclein takes a central part in aetiology and pathogenesis of Parkinson's disease-one of the most common human neurodegenerative diseases. The pathological changes in certain other neurodegenerative diseases are also linked to changes in the metabolism and function of α-synuclein, hence comprising a new group of diseases-synucleinopathies. The molecular and cellular mechanisms that are involved in the development of neurodegeneration in synucleinopathies are still largely unknown. As a result, the therapeutic approaches to the treatment of synucleinopathies are inadequately tampered. The development of models of neurodegenerative process in laboratory animals plays a crucial role in the study of these molecular mechanisms. Recently a special emphasis was placed on transgenic animal models with modified expression of genes, whose mutations are associated with inherited forms of human neurodegenerative diseases. The current review is devoted to the analysis of different models of synucleinopathies as a result of genetic modifications of α-synuclein expression.
Synuclein, transgenic animals, neurodegeneration, synucleinopathy, Parkinson's disease