Mitochondrial Respiration Is Impaired during Late-Stage Hamster Prion Infection.

ABSTRACT: Mitochondria are crucial to proper neuronal function and overall brain health. Mitochondrial dysfunction within the brain has been observed in many neurodegenerative diseases, including prion disease. Several markers of decreased mitochondrial activity during prion infection have been reported, yet the bioenergetic respiratory status of mitochondria from prion-infected animals is unknown. Here we show that clinically ill transgenic mice overexpressing hamster prion protein (Tg7) infected with the hamster prion strain 263K suffer from a severe deficit in mitochondrial oxygen consumption in response to the respiratory complex II substrate succinate. Characterization of the mitochondrial proteome of purified brain mitochondria from infected and uninfected Tg7 mice showed significant differenc

SUBMITTER: Faris R 

PROVIDER: S-EPMC5571259 | biostudies-literature | 2017 Sep

REPOSITORIES: biostudies-literature