Exercise Training and Neurodegeneration in Mitochondrial Disorders: Insights From the Harlequin Mouse.

ABSTRACT:

Aim

Cerebellar neurodegeneration is a main phenotypic manifestation of mitochondrial disorders caused by apoptosis-inducing factor (AIF) deficiency. We assessed the effects of an exercise training intervention at the cerebellum and brain level in a mouse model (Harlequin, Hq) of AIF deficiency.

Methods

Male wild-type (WT) and Hq mice were assigned to an exercise (Ex) or control (sedentary [Sed]) group (n = 10-12/group). The intervention (aerobic and resistance exercises) was initiated upon the first symptoms of ataxia in Hq mice (∼3 months on average) and lasted 8 weeks. Histological and biochemical analyses of the cerebellum were performed at the end of the training program to assess indicators of mitochondrial deficiency, neuronal death, oxidati

SUBMITTER: Fernandez-de la Torre M 

PROVIDER: S-EPMC7752860 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature