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001-es BibID:	BIBFORM063679
Első szerző:	Peters, Owen M.
Cím:	Gamma-synuclein pathology in amyotrophic lateral sclerosis / Owen M. Peters, Tatyana Shelkovnikova, John Robin Highley, Johnathan Cooper-Knock, Tibor Hortobagyi, Claire Troakes, Natalia Ninkina1, Vladimir L. Buchman
Dátum:	2015
ISSN:	2328-9503
Megjegyzések:	sclerosis (ALS) is the presence of intracellular inclusions in degenerating neuronsand their axons. The appearance and localization of these pathologicalstructures depend on an aggregated protein that forms their scaffold. We investigatedif c-synuclein, an aggregation-prone protein highly expressed in healthymotor neurons, and predominantly localized in their axons and synaptic terminalsis involved in ALS pathology. Methods: Immunostaining of histologicalsections and sequential protein extraction from postmortem neural samplesfollowed by immunoblotting. Results: Immunohistochemical screening revealeda subset of sporadic (9 of 31) and familial (8 of 23) ALS cases with a novel typeof pathology characterized by the accumulation of c-synuclein in distinctprofiles within the dorsolateral column. Sequential fractionation of proteinsfrom the spinal cord tissues revealed detergent-insoluble c-synuclein speciesspecifically in the dorsolateral corticospinal tracts of a ALS patient withc-synuclein-positive profiles in this region. These profiles are negative for proteinmarkers commonly found in pathological inclusions in the spinal cord ofALS patients and most probably represent degenerated axons of upper motorneurons that have lost their neurofilaments. A subset of these profiles wasfound in association with phagocytic cells positive for Mac-2/Galectin-3. Asmaller subset of studied ALS cases (4 of 54) contained large cytoplasmic inclusionsin the cell body of remaining spinal motor neurons. Interpretation: Ourobservations suggest that pathological aggregation of c-synuclein might contributeto the pathogenesis of ALS.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:	Annals of Clinical and Translational Neurology. - 2 : 1 (2015), p. 29-37. -
További szerzők:	Shelkovnikova, Tatyana Highley, John Robin Cooper-Knock, Johnathan Hortobágyi Tibor (1965-) (patológus) Troakes, Claire Ninkina, Natalia Buchman, Vladimir L.
Internet cím:	Szerző által megadott URL DOI Intézményi repozitóriumban (DEA) tárolt változat
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