title: Mitochondrial homeostasis molecules: regulation by a trio of recessive Parkinson's disease genes.
abstract: Mitochondria are small organelles that produce the majority of cellular energy as ATP. Mitochondrial dysfunction has been implicated in the pathogenesis of Parkinson's disease (PD), and rare familial forms of PD provide valuable insight into the pathogenic mechanism underlying mitochondrial impairment, even though the majority of PD cases are sporadic. The regulation of mitochondria is crucial for the maintenance of energy-demanding neuronal functions in the brain. Mitochondrial biogenesis and mitophagic degradation are the major regulatory pathways that preserve optimal mitochondrial content, structure and function. In this mini-review, we provide an overview of the mitochondrial quality control mechanisms, emphasizing regulatory molecules in mitophagy and biogenesis that specifically interact with the protein products of three major recessive familial PD genes, PINK1, Parkin and DJ-1.
authors.affiliation: Department of Brain and Cognitive Sciences, Brain Disease Research Institute, Ewha Womans University, Seoul 120-750, Korea.
authors.forename: Ji-Young
authors.initials: JY
authors.lastname: Han
authors.affiliation: Graduate School of Pharmaceutical Sciences, College of Pharmacy, Ewha Womans University, Seoul 120-750, Korea.
authors.forename: Ji-Soo
authors.initials: JS
authors.lastname: Kim
authors.affiliation: Department of Brain and Cognitive Sciences, Brain Disease Research Institute, Ewha Womans University, Seoul 120-750, Korea. ; Graduate School of Pharmaceutical Sciences, College of Pharmacy, Ewha Womans University, Seoul 120-750, Korea.
authors.forename: Jin H
authors.initials: JH
authors.lastname: Son
keywords: Biogenesis; DJ-1; Mitophagy; PD genes; PINK1; Parkin