title: Complex I deficiencies in neurological disorders.
abstract: Complex I is the point of entry in the mitochondrial electron transport chain for NADH reducing equivalents, and it behaves as a regulatable pacemaker of respiratory ATP production in human cells. Defects in complex I are associated with several human neurological disorders, including primary mitochondrial diseases, Parkinson disease (PD), and Down syndrome, and understanding the activity and regulation of complex I may reveal aspects of the underlying pathogenic mechanisms. Complex I is regulated by cyclic AMP (cAMP) and the protein kinase A (PKA) signal transduction pathway, and elucidating the role of the cAMP/PKA system in regulating complex I and oxygen free radical production provides new perspectives for devising therapeutic strategies for neurological diseases.
authors.affiliation: Institute of Biomembranes and Bioenergetics (IBBE), Consiglio Nazionale delle Ricerche, Bari, Italy. sergio.papa@uniba.it
authors.forename: Sergio
authors.initials: S
authors.lastname: Papa
authors.forename: Domenico
authors.initials: D
authors.lastname: De Rasmo
mesh_terms: D000242:Cyclic AMP / Q000378:metabolism; D004314:Down Syndrome / Q000235:genetics / Q000378:metabolism; D042967:Electron Transport Complex I / Q000172:deficiency*; D006801:Humans; D028361:Mitochondrial Diseases / Q000235:genetics / Q000378:metabolism; D009422:Nervous System Diseases / Q000235:genetics* / Q000378:metabolism*; D010085:Oxidative Phosphorylation; D010300:Parkinson Disease / Q000235:genetics / Q000378:metabolism; D017382:Reactive Oxygen Species / Q000378:metabolism
chemical_list: D017382:Reactive Oxygen Species; D000242:Cyclic AMP; D042967:Electron Transport Complex I