TigarB causes mitochondrial dysfunction and neuronal loss in PINK1 deficiency.

ABSTRACT:

Objective

Loss of function mutations in PINK1 typically lead to early onset Parkinson disease (PD). Zebrafish (Danio rerio) are emerging as a powerful new vertebrate model to study neurodegenerative diseases. We used a pink1 mutant (pink(-/-) ) zebrafish line with a premature stop mutation (Y431*) in the PINK1 kinase domain to identify molecular mechanisms leading to mitochondrial dysfunction and loss of dopaminergic neurons in PINK1 deficiency.

Methods

The effect of PINK1 deficiency on the number of dopaminergic neurons, mitochondrial function, and morphology was assessed in both zebrafish embryos and adults. Genome-wide gene expression studies were undertaken to identify novel pathogenic mechanisms. Functional experiments were carried out to further investigate the effect

SUBMITTER: Flinn LJ 

PROVIDER: S-EPMC4154126 | biostudies-literature | 2013 Dec

REPOSITORIES: biostudies-literature