About: Detection of the GPI-anchorless prion protein fragment PrP226 in human brain     Goto   Sponge   NotDistinct   Permalink

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Description
  • Background: The accumulation of the misfolded forms of cellular prion protein, i.e. prions (PrPSc), in the brain is one of the crucial characteristics of fatal neurodegenerative disorders, called transmissible spongiform encephalopathies (TSEs). Cellular prion protein is normally linked to the cell surface by the glycosylphosphatidylinositol (GPI) anchor. There is accumulating evidence that the GPI-anchorless prion protein may act as an accelerator of formation and propagation of prions. In the TSE affected human brain we have previously discovered a novel GPI-anchorless prion protein fragment, named PrP226*, which ends with the tyrosine 226. This fragment can be labeled specifically by the monoclonal antibody V5B2. Methods: We developed a DELFIA based assay for quick and sensitive detection of the PrP226* fragment in human brain tissue homogenates. By calculating the ratio between the signals of native (N) and denatured (D) samples applied to the assay we were able to observe significant difference between 24 TSE affected brains and 10 control brains. The presence of PrP226* in brain tissue was confirmed by western blot. Results: Our results demonstrate that PrP226* is present in small quantities in healthy human brain, whereas in degenerated brain it accumulates in prion aggregates, proportionally to PrPSc. Samples with high D/N ratio generally comprised more proteinase K resistant PrP, while no correlation was found between the quantity of PrP226* and standard classification of Creutzfeldt-Jakob disease (CJD).
  • Background: The accumulation of the misfolded forms of cellular prion protein, i.e. prions (PrPSc), in the brain is one of the crucial characteristics of fatal neurodegenerative disorders, called transmissible spongiform encephalopathies (TSEs). Cellular prion protein is normally linked to the cell surface by the glycosylphosphatidylinositol (GPI) anchor. There is accumulating evidence that the GPI-anchorless prion protein may act as an accelerator of formation and propagation of prions. In the TSE affected human brain we have previously discovered a novel GPI-anchorless prion protein fragment, named PrP226*, which ends with the tyrosine 226. This fragment can be labeled specifically by the monoclonal antibody V5B2. Methods: We developed a DELFIA based assay for quick and sensitive detection of the PrP226* fragment in human brain tissue homogenates. By calculating the ratio between the signals of native (N) and denatured (D) samples applied to the assay we were able to observe significant difference between 24 TSE affected brains and 10 control brains. The presence of PrP226* in brain tissue was confirmed by western blot. Results: Our results demonstrate that PrP226* is present in small quantities in healthy human brain, whereas in degenerated brain it accumulates in prion aggregates, proportionally to PrPSc. Samples with high D/N ratio generally comprised more proteinase K resistant PrP, while no correlation was found between the quantity of PrP226* and standard classification of Creutzfeldt-Jakob disease (CJD). (en)
Title
  • Detection of the GPI-anchorless prion protein fragment PrP226 in human brain
  • Detection of the GPI-anchorless prion protein fragment PrP226 in human brain (en)
skos:prefLabel
  • Detection of the GPI-anchorless prion protein fragment PrP226 in human brain
  • Detection of the GPI-anchorless prion protein fragment PrP226 in human brain (en)
skos:notation
  • RIV/00064190:_____/13:#0000663!RIV14-MZ0-00064190
http://linked.open...avai/riv/aktivita
http://linked.open...avai/riv/aktivity
  • I, P(MEB091004), P(NT14145), S
http://linked.open...iv/cisloPeriodika
  • 126
http://linked.open...vai/riv/dodaniDat
http://linked.open...aciTvurceVysledku
http://linked.open.../riv/druhVysledku
http://linked.open...iv/duvernostUdaju
http://linked.open...titaPredkladatele
http://linked.open...dnocenehoVysledku
  • 68747
http://linked.open...ai/riv/idVysledku
  • RIV/00064190:_____/13:#0000663
http://linked.open...riv/jazykVysledku
http://linked.open.../riv/klicovaSlova
  • Transmissible spongiform encephalopathies; Creutzfeldt-Jakob disease; GSS; Prion; V5B2; Immunoassay; DELFIA; Anchorless PrP; PrP fragment; Proteinase K (en)
http://linked.open.../riv/klicoveSlovo
http://linked.open...odStatuVydavatele
  • GB - Spojené království Velké Británie a Severního Irska
http://linked.open...ontrolniKodProRIV
  • [61F214F1C152]
http://linked.open...i/riv/nazevZdroje
  • BMC NEUROLOGY
http://linked.open...in/vavai/riv/obor
http://linked.open...ichTvurcuVysledku
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http://linked.open...vavai/riv/projekt
http://linked.open...UplatneniVysledku
http://linked.open...v/svazekPeriodika
  • 13
http://linked.open...iv/tvurceVysledku
  • Matěj, Radoslav
http://linked.open...ain/vavai/riv/wos
  • 000324882600002
issn
  • 1471-2377
number of pages
http://bibframe.org/vocab/doi
  • 10.1186/1471-2377-13-126
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