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Bioenergetic dysfunction in Huntington's disease human cybrids

Ferreira, I. Luísa; Cunha-Oliveira, Teresa; Nascimento, Maria V.; Ribeiro, Márcio; Proença, M. Teresa; Januário, Cristina; Oliveira, Catarina R.

In this work we studied the mitochondrial-associated metabolic pathways in Huntington's disease (HD) versus control (CTR) cybrids, a cell model in which the contribution of mitochondrial defects from patients is isolated. HD cybrids exhibited an interesting increase in ATP levels, when compared to CTR cybrids. Concomitantly, we observed increased glycolytic rate in HD cybrids, as revealed by increased lactate/p...


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