Alpha-synuclein overexpression promotes aggregation of mutant huntingtin.
Protein aggregates are a neuropathological feature of Huntington's disease and Parkinson's disease. Mutant huntingtin exon 1 with 72 CAG repeats fused to enhanced green fluorescent protein (EGFP) forms hyperfluorescent inclusions in PC12 cells. Inclusion formation is enhanced in cells co-transfected with EGFP-huntingtin-(CAG)(72) and alpha-synuclein, a major component of Parkinson's disease aggregates. However, alpha-synuclein does not form aggregates by itself, nor does it appear in huntingtin inclusions in vitro.
Furlong
R A
RA
Department of Medical Genetics, Cambridge University, Wellcome Trust Centre for the Study of Molecular Mechanisms in Disease, Cambridge Institute for Medical Research, Wellcome/MRC Building, Addenbrooke's Hospital, Hills Road, Cambridge CB2 2XY, U.K.
Narain
Y
Y
Rankin
J
J
Wyttenbach
A
A
Rubinsztein
D C
DC
D000818:Animals; D001483:Base Sequence; D017931:DNA Primers; D049452:Green Fluorescent Proteins; D000071058:Huntingtin Protein; D007150:Immunohistochemistry; D008164:Luminescent Proteins / Q000235:genetics; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000378:metabolism*; D009687:Nuclear Proteins / Q000235:genetics / Q000378:metabolism*; D016716:PC12 Cells; D051381:Rats; D011993:Recombinant Fusion Proteins / Q000235:genetics; D051843:Synucleins; D014162:Transfection; D051844:alpha-Synuclein
D017931:DNA Primers; C499524:Htt protein, rat; D000071058:Huntingtin Protein; D008164:Luminescent Proteins; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins; D011993:Recombinant Fusion Proteins; C497606:Snca protein, rat; D051843:Synucleins; D051844:alpha-Synuclein; D049452:Green Fluorescent Proteins