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@ARTICLE{VillarPiqu:830503,
author = {Villar-Piqué, Anna and Rossetti, Giulia and Ventura,
Salvador and Carloni, Paolo and Fernández, Claudio O. and
Outeiro, Tiago Fleming},
title = {{C}opper({II}) and the pathological {H}50{Q} α-synuclein
mutant: {E}nvironment meets genetics},
journal = {Communicative $\&$ integrative biology},
volume = {10},
number = {1},
issn = {1942-0889},
address = {Austin, Tex.},
publisher = {Landes Bioscience},
reportid = {FZJ-2017-04044},
pages = {e1270484 -},
year = {2017},
abstract = {Copper is one of the metals described to bind the Parkinson
disease-related protein α-synuclein (aSyn), and to promote
its aggregation. Although histidine at position 50 in the
aSyn sequence is one of the most studied copper-anchoring
sites, its precise role in copper binding and aSyn
aggregation is still unclear. Previous studies suggested
that this residue does not significantly affect
copper-mediated aSyn aggregation. However, our findings
showed that the aggregation of the pathological H50Q aSyn
mutant is enhanced by copper hints otherwise. Despite the
inexistence of a model for aSyn H50Q-copper complexation, we
discuss possible mechanisms by which this metal contributes
to the misfolding and self-assembly of this particular aSyn
mutant. Considering the genetic association of the H50Q
mutation with familial forms of Parkinson disease, and the
fact that copper homeostasis is deregulated in this
disorder, understanding the interplay between both factors
will shed light into the molecular and cellular mechanisms
triggering the development and spreading of the aSyn
pathology.},
cin = {IAS-5 / INM-9 / JSC},
ddc = {570},
cid = {I:(DE-Juel1)IAS-5-20120330 / I:(DE-Juel1)INM-9-20140121 /
I:(DE-Juel1)JSC-20090406},
pnm = {572 - (Dys-)function and Plasticity (POF3-572) / 511 -
Computational Science and Mathematical Methods (POF3-511)},
pid = {G:(DE-HGF)POF3-572 / G:(DE-HGF)POF3-511},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:28289488},
doi = {10.1080/19420889.2016.1270484},
url = {https://juser.fz-juelich.de/record/830503},
}