Alpha-synuclein and transgenic mouse models.
Identified as the cause of some familial forms of Parkinson disease (PD) and as one of the major component of Lewy bodies, alpha-synuclein (alpha-syn) became the molecular hallmark of several neurodegenerative conditions now designated as synucleinopathies. Transgenic models have been generated to elucidate its physiological and pathologic roles. Although none of the lines created display dopaminergic neuronal death in the substantia nigra, the models recapitulate some features of synucleinopathies and are useful to study the potential pathogenic role of alpha-synuclein and its molecular partners. This review describes the different alpha-synuclein transgenic models, their clinical relevance to synucleinopathies, and their further utilization to understand the disease process.
Departments of Neurology and Neurobiology, The David Geffen School of Medicine at UCLA, Los Angeles, CA 90095-1769, USA.
Pierre-Olivier
PO
Fernagut
Marie-Françoise
MF
Chesselet
D000818:Animals; D004195:Disease Models, Animal; D051379:Mice; D008822:Mice, Transgenic; D009419:Nerve Tissue Proteins / Q000378:metabolism*; D019636:Neurodegenerative Diseases / Q000209:etiology* / Q000378:metabolism* / Q000503:physiopathology; D051843:Synucleins; D051844:alpha-Synuclein
D009419:Nerve Tissue Proteins; C497605:Snca protein, mouse; D051843:Synucleins; D051844:alpha-Synuclein