Mutations in the DJ-1 gene are associated with rare forms of autosomal recessive early-onset PD (172). Mice with knock-out of DJ-1 appear hypoactive, show no apparent loss of midbrain dopaminergic neurons, but do display altered electrophysiological properties in striatal neurons that can be rescued by targeting dopamine receptor D1 (173). Analysis of the structure, function, and pattern of expression of DJ-1 in mice, Drosophila, and in cell culture indicate that DJ-1 protein interacts with mitochondria playing a role in oxidative stress and apoptosis (174,175). DJ-1 has been shown to interact with a number of other proteins implicated in familial parkinsonism, including alpha-synuclein, thus regulating its function and potential toxicity (176).
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