Analysis of the molecular mechanism by which the genes for early‐onset parkinson's disease regulate mitochondrial maintenance

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Mitochondrial dysregulation is now implicated in various human diseases including cancer, diabetes, myopathy and neurodegeneration. The pathological hallmark of the second most common neurodegenerative disorder PDis the progressive degeneration of dopaminergic neurons in the midbrain.A series of elegant studies have demonstrated that two Parkinson’s disease(PD)-associated genes PINK1and parkinare involved in the maintenance of healthy mitochondria. Parkin in co-operation with PINK1 specifically recognises damaged mitochondria with reduced mitochondrial membrane potential (Δψm), rapidly isolates them from the mitochondrial network and eliminates them through the ubiquitin-proteasome and autophagy pathways. Our studies combined with Drosophilagenetics and cell biologycontributedto understanding the molecular mechanisms of PINK1 and Parkin-mediated mitochondrial regulation and the pathological mechanisms how defects in the PINK1-Parkin pathway leads toneurodegeneration in PD.