A cybrid cell model for the assessment of the link between mitochondrial deficits and sporadic Parkinson's disease
DM Arduíno, AR Esteves, RH Swerdlow… - … Medicine: Volume II …, 2015 - Springer
DM Arduíno, AR Esteves, RH Swerdlow, SM Cardoso
Mitochondrial Medicine: Volume II, Manipulating Mitochondrial Function, 2015•SpringerParkinson'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 …
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 …
Abstract
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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