title: Autophagy and its possible roles in nervous system diseases, damage and repair.
abstract: Increased numbers of autophagosomes/autophagic vacuoles are seen in a variety of physiological and pathological states in the nervous system. In many cases, it is unclear if this phenomenon is the result of increased autophagic activity or decreased autophagosome-lysosome fusion. The functional significance of autophagy and its relationship to cell death in the nervous system is also poorly understood. In this review, we have considered these issues in the context of acute neuronal injury and a range of chronic neurodegenerative conditions, including the Lurcher mouse, Alzheimer's, Parkinson's, Huntington's and prion diseases. While many issues remain unresolved, these conditions raise the possibility that autophagy can have either deleterious or protective effects depending on the specific situation and stage in the pathological process.
authors.affiliation: Departments of Medical Genetics, University of Cambridge, Cambridge Institute for Medical Research, Wellcome Trust/MRC Building, Addenbrooke's Hospital, Hills Road, Cambridge, UK. dcr1000@cus.cam.ac.uk
authors.forename: David C
authors.initials: DC
authors.lastname: Rubinsztein
authors.forename: Marian
authors.initials: M
authors.lastname: DiFiglia
authors.forename: Nathaniel
authors.initials: N
authors.lastname: Heintz
authors.forename: Ralph A
authors.initials: RA
authors.lastname: Nixon
authors.forename: Zheng-Hong
authors.initials: ZH
authors.lastname: Qin
authors.forename: Brinda
authors.initials: B
authors.lastname: Ravikumar
authors.forename: Leonidas
authors.initials: L
authors.lastname: Stefanis
authors.forename: Aviva
authors.initials: A
authors.lastname: Tolkovsky
mesh_terms: D000544:Alzheimer Disease / Q000473:pathology*; D000818:Animals; D001343:Autophagy / Q000502:physiology*; D006801:Humans; D051379:Mice; D008818:Mice, Neurologic Mutants; D009474:Neurons / Q000473:pathology*; D010300:Parkinson Disease / Q000473:pathology*; D010455:Peptides / Q000378:metabolism; D010588:Phagosomes / Q000473:pathology*; D017096:Prion Diseases / Q000473:pathology*
chemical_list: D010455:Peptides; C097188:polyglutamine