title: Mitochondrial quality control: a matter of life and death for neurons.
abstract: Neuronal survival critically depends on the integrity and functionality of mitochondria. A hierarchical system of cellular surveillance mechanisms protects mitochondria against stress, monitors mitochondrial damage and ensures the selective removal of dysfunctional mitochondrial proteins or organelles. Mitochondrial proteases emerge as central regulators that coordinate different quality control (QC) pathways within an interconnected network of mechanisms. A failure of this system causes neuronal loss in a steadily increasing number of neurodegenerative disorders, which include Parkinson's disease, spinocerebellar ataxia, spastic paraplegia and peripheral neuropathies. Here, we will discuss the role of the mitochondrial QC network for neuronal survival and neurodegeneration.
authors.affiliation: Institute for Zoology, University of Cologne, Cologne, Germany. elena.rugarli@uni-koeln.de
authors.forename: Elena I
authors.initials: EI
authors.lastname: Rugarli
authors.forename: Thomas
authors.initials: T
authors.lastname: Langer
mesh_terms: D000818:Animals; D017209:Apoptosis / Q000502:physiology*; D002470:Cell Survival / Q000502:physiology*; D006801:Humans; D008928:Mitochondria / Q000378:metabolism*; D024101:Mitochondrial Proteins / Q000378:metabolism; D019636:Neurodegenerative Diseases / Q000378:metabolism / Q000473:pathology; D009474:Neurons / Q000166:cytology* / Q000378:metabolism / Q000473:pathology; D010447:Peptide Hydrolases / Q000378:metabolism
chemical_list: D024101:Mitochondrial Proteins; D010447:Peptide Hydrolases