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Article

Assessing Mitochondrial Function in In Vitro and Ex Vivo Models of Huntington's Disease.

Methods in molecular biology (Clifton, N.J.) - 1 Jan 2018

Ferreira I Luísa, Carmo Catarina, Naia Luana, I Mota Sandra, Cristina Rego A

Abstract excerpt

Mitochondrial dysfunction has gained a preponderant role in the pathogenesis of Huntington's disease (HD). Mutant huntingtin (mHTT) directly interacts with mitochondria in a deleterious manner. As the central hub of the cell, not only mitochondrial bioenergetics is affected but there is also diminished mitochondrial membrane potential (Δψ m) and altered production of reactive oxygen species (ROS). Restoration of...

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PMID: 29856029
DOI: 10.1007/978-1-4939-7825-0_19

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