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Arduino, D.M. ; Esteves, A.R.* ; Swerdlow, R.H.* ; Cardoso, S.M.*

A cybrid cell model for the assessment of the link between mitochondrial deficits and sporadic Parkinson's disease.

Methods Mol. Biol. 1265, 415-424 (2015)
DOI
Open Access Green möglich sobald Postprint bei der ZB eingereicht worden ist.
Parkinson's disease (PD) is a multifactorial and clinically complex age-related movement disorder. The cause of its most common form (sporadic PD, sPD) is unknown, but one prominent causal factor is mitochondrial dysfunction. Although several genetic- and toxin-based models have been developed along the last decades to mimic the pathological cascade of PD, cellular models that reliably recapitulate the pathological features of the neurons that degenerate in PD are scarce. We describe here the generation of cytoplasmic hybrid cells (or cybrids) as a cellular model of sPD. This approach consists on the fusion of platelets harboring mtDNA from sPD patients with cells in which the endogenous mtDNA has been depleted (Rho0 cells). The sPD cybrid model has been successful in recapitulating most of the hallmarks of sPD, constituting now a validated model for addressing the link between mitochondrial dysfunction and sPD pathology.
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Publikationstyp Artikel: Journalartikel
Dokumenttyp Wissenschaftlicher Artikel
Korrespondenzautor
Schlagwörter Alpha-synuclein Oligomers ; Cellular Models ; Cybrids ; Mitochondria ; Mitochondrial Impairment ; Mtdna ; Neurodegeneration ; Oxidative Stress ; Parkinson's Disease ; Rho Cells
ISSN (print) / ISBN 1064-3745
e-ISSN 1940-6029
Konferenztitel Mitochondrial Medicine
Quellenangaben Band: 1265, Heft: , Seiten: 415-424 Artikelnummer: , Supplement: ,
Verlag Springer
Verlagsort Berlin [u.a.]
Nichtpatentliteratur Publikationen
Begutachtungsstatus Peer reviewed