The<i>Polg</i>Mutator Phenotype Does Not Cause Dopaminergic Neurodegeneration in<i>DJ-1</i>-Deficient Mice

Abstract excerpt

Mutations in the DJ-1 gene cause autosomal recessive parkinsonism in humans. Several mouse models of DJ-1 deficiency have been developed, but they do not have dopaminergic neuron cell death in the substantia nigra pars compacta (SNpc). Mitochondrial DNA (mtDNA) damage occurs frequently in the aged human SNpc but not in the mouse SNpc. We hypothesized that the reason DJ-1-deficient mice do not have dopaminergic...