title: Ubiquitinated inclusions and neuronal cell death.
abstract: Ubiquitinated inclusions and selective neuronal cell death are considered the pathological hallmarks of Parkinson's disease and other neurodegenerative diseases. Recent genetic, pathological and biochemical evidence suggests that dysfunction of ubiquitin-dependent protein degradation by the proteasome might be a contributing, if not initiating factor in the pathogenesis of these diseases. In neuronal cell culture models inhibition of the proteasome leads to cell death and formation of fibrillar ubiquitin and alpha-synuclein-positive inclusions, thus modeling some aspects of Lewy body diseases. The processes of inclusion formation and neuronal cell death share some common mechanisms, but can also be dissociated at a certain level.
authors.affiliation: Department of Neurology, Columbia University, New York, NY 10032, USA.
authors.forename: I
authors.initials: I
authors.lastname: Lang-Rollin
authors.forename: H
authors.initials: H
authors.lastname: Rideout
authors.forename: L
authors.initials: L
authors.lastname: Stefanis
mesh_terms: D000544:Alzheimer Disease / Q000473:pathology; D000818:Animals; D016923:Cell Death / Q000235:genetics / Q000502:physiology; D006801:Humans; D002479:Inclusion Bodies / Q000235:genetics / Q000378:metabolism / Q000502:physiology*; D009419:Nerve Tissue Proteins / Q000096:biosynthesis / Q000235:genetics; D019636:Neurodegenerative Diseases / Q000473:pathology; D009474:Neurons / Q000473:pathology / Q000502:physiology*; D010300:Parkinson Disease / Q000473:pathology; D051843:Synucleins; D014451:Ubiquinone / Q000378:metabolism*; D051844:alpha-Synuclein
chemical_list: D009419:Nerve Tissue Proteins; C497604:SNCA protein, human; D051843:Synucleins; D051844:alpha-Synuclein; D014451:Ubiquinone