title: [ATP-dependent proteases in the quality control of mitochondrial proteome].
abstract: Mitochondria play the fundamental role in energy production and integration of many important metabolic and signalling pathways, which makes them essential for the function of a cell. The optimal operation of mitochondria depends on the qualitative and quantitative composition of the organellar proteins - the proteome. To maintain the homeostasis of the mitochondrial proteome, mitochondria developed a protein quality control system, which acts on the molecular, cellular and organellar levels. ATP-dependent proteases constitute a key element of this system. It consists of Lon/PIM1 and ClpXP proteases located in the mitochondrial matrix as well as AAA proteases anchored in the inner mitochondrial membrane. The ATP-dependent proteases degrade misfolded, damaged or not assembled proteins. These enzymes are also involved in complex regulatory mechanisms such as mitochondrial translation, fusion and response to stress. Lack of any of ATP-dependent proteases leads to mitochondrial dysfunction and the development of many major diseases in humans. This work summarizes the current knowledge of the ATP-dependent proteolytic system in mitochondria in different organisms.
authors.affiliation: University of Wroclaw, Faculty of Biotechnology, 14A F. Joliot-Curie St., 50-383 Wroclaw, Poland.
authors.forename: Marta
authors.initials: M
authors.lastname: Kołodziejczak
authors.affiliation: University of Wroclaw, Faculty of Biotechnology, 14A F. Joliot-Curie St., 50-383 Wroclaw, Poland.
authors.forename: Magdalena
authors.initials: M
authors.lastname: Opalińska
authors.affiliation: University of Wroclaw, Faculty of Biotechnology, 14A F. Joliot-Curie St., 50-383 Wroclaw, Poland.
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Czarna
authors.affiliation: University of Wroclaw, Faculty of Biotechnology, 14A F. Joliot-Curie St., 50-383 Wroclaw, Poland.
authors.forename: Hanna
authors.initials: H
authors.lastname: Jańska
mesh_terms: D049069:ATP-Dependent Proteases / Q000378:metabolism*; D056890:Eukaryota / Q000378:metabolism; D006801:Humans; D008928:Mitochondria / Q000378:metabolism*; D024101:Mitochondrial Proteins / Q000378:metabolism; D020543:Proteome / Q000378:metabolism*
chemical_list: D024101:Mitochondrial Proteins; D020543:Proteome; D049069:ATP-Dependent Proteases
keywords: AAA protease; ClpXP; FTSH; Lon; mitochondrial proteases; protein quality control in mitochondria