title: Pathogenic mechanisms of neurodegeneration in Parkinson disease.
abstract: The last 2 decades represent a period of unparalleled advancement in the understanding of the pathogenesis of Parkinson disease (PD). The discovery of several forms of familial parkinsonism with mendelian inheritance has elucidated insights into the mechanisms underlying the degeneration of dopaminergic neurons of the substantia nigra that histologically characterize PD. α-Synuclein, the principal component of Lewy bodies, remains the presumed pathogen at the heart of the current model; however, concurrently, a diverse range of other mechanisms have been implicated. The creation of a coherent disease model will be crucial to the development of effective disease modifying therapies for sporadic PD.
authors.affiliation: Department of Clinical Neurosciences, UCL Institute of Neurology, Rowland Hill Street, Hampstead, London NW3 2PF, UK.
authors.forename: Stephen
authors.initials: S
authors.lastname: Mullin
authors.affiliation: Department of Clinical Neurosciences, UCL Institute of Neurology, Rowland Hill Street, Hampstead, London NW3 2PF, UK. Electronic address: a.schapira@ucl.ac.uk.
authors.forename: Anthony H V
authors.initials: AH
authors.lastname: Schapira
mesh_terms: D005962:Glucosylceramidase / Q000378:metabolism; D006801:Humans; D000071158:Leucine-Rich Repeat Serine-Threonine Protein Kinase-2; D009154:Mutation / Q000235:genetics; D009410:Nerve Degeneration / Q000209:etiology*; D010300:Parkinson Disease / Q000150:complications* / Q000235:genetics*; D017346:Protein Serine-Threonine Kinases; D013378:Substantia Nigra / Q000378:metabolism / Q000473:pathology; D051844:alpha-Synuclein / Q000235:genetics / Q000378:metabolism*
chemical_list: C497604:SNCA protein, human; D051844:alpha-Synuclein; C495280:LRRK2 protein, human; D000071158:Leucine-Rich Repeat Serine-Threonine Protein Kinase-2; D017346:Protein Serine-Threonine Kinases; D005962:Glucosylceramidase
keywords: Lewy bodies; Mitochondrial dysfunction; Parkinson disease; α-Synuclein