Modulation of alpha-synuclein expression in transgenic animals for modelling synucleinopathies--is the juice worth the squeeze?
Studies of various animal models have made a substantial contribution to the recent progress in understanding of molecular and cellular bases of neurodegenerative disorders. Modelling of neurodegeneration by genetic alteration of laboratory animals became one of the most powerful tools of modern experimental neurology. The crucial event in pathogenesis of neurodegenerative diseases known as synucleinopathies is modification of alpha-synuclein metabolism caused by missense mutations, increased expression of the gene, or impaired degradation or intracellular compartmentalisation of the protein. Therefore, manipulations with expression of alpha-synuclein in laboratory animals were widely used for creating models of these diseases. In the present review we discuss strong and weak sides of such models, what has been already learned from studies of these animals and what types of models might be useful to further our knowledge about pathogenesis of different synucleinopathies.
Buchman
Vladimir L
VL
School of Biosciences, Cardiff University, Museum Avenue, Cardiff, CF10 3US, UK. buchmanvl@cf.ac.uk
Ninkina
Natalia
N
D000818:Animals; D030801:Animals, Genetically Modified / Q000235:genetics / Q000378:metabolism; D002451:Cell Compartmentation; D004195:Disease Models, Animal; D006801:Humans; D051379:Mice; D020125:Mutation, Missense; D019636:Neurodegenerative Diseases / Q000209:etiology*; D009474:Neurons / Q000378:metabolism; D009836:Oligodendroglia / Q000378:metabolism; D051844:alpha-Synuclein / Q000235:genetics / Q000378:metabolism*
D051844:alpha-Synuclein