title: Mitochondrial quality control by the Pink1/Parkin system.
abstract: Mitochondrial dysfunction represents a prominent pathological feature in many neurodegenerative diseases, particularly in Parkinson's disease (PD). Mutations in the genes encoding the proteins Pink1 and Parkin have been identified as genetic risk factors in familiar cases of PD. Research during the last decade has identified both proteins as crucial components of an organellar quality control system that contributes to the maintenance of mitochondrial function in healthy cells. The Pink1/Parkin system acts as a sensor for mitochondrial quality and is activated, in particular, after the loss of the electric potential across the inner mitochondrial membrane. Pink1 molecules accumulate at the surface of damaged mitochondria to recruit and activate Parkin, which, in turn, elicits a signaling pathway eventually leading to the autophagic removal of the damaged organelles. This review summarizes recent advances in our knowledge of the functional role of the Pink1/Parkin system in preventing the accumulation of damaged mitochondria by mitophagy.
authors: Institut für Biochemie und Molekularbiologie (IBMB), Universität Bonn, Nussallee 11, 53115, Bonn, Germany.
authors: Cornelia
authors: C
authors: Rüb
authors: Institut für Biochemie und Molekularbiologie (IBMB), Universität Bonn, Nussallee 11, 53115, Bonn, Germany.
authors: Anne
authors: A
authors: Wilkening
authors: Institut für Biochemie und Molekularbiologie (IBMB), Universität Bonn, Nussallee 11, 53115, Bonn, Germany. wolfgang.voos@uni-bonn.de.
authors: Wolfgang
authors: W
authors: Voos
mesh_terms: D000818:Animals; D006801:Humans; D008928:Mitochondria / Q000378:metabolism*; D063306:Mitophagy; D008954:Models, Biological; D011494:Protein Kinases / Q000378:metabolism*; D015398:Signal Transduction; D044767:Ubiquitin-Protein Ligases / Q000378:metabolism*; D000099202:PTEN-Induced Putative Kinase
chemical_list: D011494:Protein Kinases; D044767:Ubiquitin-Protein Ligases; D000099202:PTEN-Induced Putative Kinase; C111567:parkin protein
keywords: Mitochondria; Mitophagy; Organellar quality control; Parkin; Pink1