title: Mitochondrial dynamics in aging and disease.
abstract: Mitochondria are self-replicating organelles but nevertheless strongly depend on supply coded in nuclear genes. They serve many physiological demands in living cells. Supply of the cytoplasm with ATP and engagement in Ca(2+) regulation belong to the main functions of mitochondria. In large eukaryotic cells, in particular in neurons, with their long dendrites and axons, mitochondria have to move to the sites of their action. This trafficking involves several motor molecules and mechanisms to sense the sites of requirements of mitochondria. With aging and as a consequence of some diseases, mitochondrial components may be rendered dysfunctional, and mtDNA mutations arise during the course of replication and by the action of reactive oxygen species. Mutants in motor molecules engaged in trafficking and in the machinery of fusion and fission are causing severe deficiencies on the cellular level; they support neurodegeneration and, thus, cause many diseases. Frequent fusion and fission events mediate the elimination of impaired parts from mitochondria which finally will be degraded by autophagosomes. Extensive fusion provides a basis for functional complementation. Mobility of proteins and small molecules within the mitochondria is necessary to reach the functional goals of fusion and fission, although cristae and a large fraction of proteins of the respiratory complexes proved to be stable for hours after fusion and perform slow exchange of material.
authors.affiliation: Institute for Cell Biology and Neurosciences, Goethe University Frankfurt am Main, Frankfurt am Main, Germany.
authors.forename: Jürgen
authors.initials: J
authors.lastname: Bereiter-Hahn
mesh_terms: D000375:Aging / Q000473:pathology*; D000818:Animals; D006801:Humans; D028361:Mitochondrial Diseases / Q000378:metabolism* / Q000473:pathology*; D063154:Mitochondrial Dynamics; D051336:Mitochondrial Membranes / Q000378:metabolism; D024101:Mitochondrial Proteins / Q000378:metabolism; D053142:Mitochondrial Size
chemical_list: D024101:Mitochondrial Proteins
keywords: Aging; Fission; Fusion; Mitochondrial dynamics; Mitochondrial trafficking; Motor molecules; Neurodegenerative diseases