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- Protease-sensitive_prionopathy abstract "Protease-sensitive prionopathy (PSPr) is a neurodegenerative disorder caused by an abnormal isoform of the prion protein. Contrary to the prions in Creutzfeldt-Jakob disease (CJD), the prions in this condition are sensitive to protease activity.PSPr was first described in an abstract for a conference on prions in 2006, and this study was published in a 2008 report on 11 cases. The study was conducted by Gambetti P. and coworkers from the United States National Prion Disease Pathology Surveillance Center.Symptoms of PSPr are behavioral and psychiatric abnormalities with progressive decline in cognitive and motor functions (including dementia, ataxia, parkinsonism, psychosis, aphasia and mood disorders). This is similar to what occurs in other spongiform encephalopathies, and the condition can be mistaken for Alzheimer's dementia. Contrary to what is the case in CJD, there were no cases with a positive 14-3-3 protein test in the cerebrospinal fluid, no periodic complexes on electroencephalography (EEG), and no pathognomonic changes on diffusion-weighted magnetic resonance images. The mean age of onset in the original 11 patients was 62 years. Patients progressed to death in 20 months on average. PSPr accounted for three percent of prion disease cases evaluated by the U.S. National Prion Disease Pathology Surveillance Center (prion diseases occur in the order of magnitude of 1 case per million people).The diagnosis can be made on pathological examination. There are unique microscopic and immunohistochemical features, and the prions cannot be digested using proteases. Because 8 out of 10 patients had a positive family history of dementia in the original study, a genetic cause was suspected. Although all cases were homozygous for valine at codon 129 of the PrP gene, no mutations were detected.How these protease-resistant prions lead to neurological disease remains incompletely understood. For instance, protein-resistant prions have been found in normal human brains.".
- Protease-sensitive_prionopathy wikiPageID "21096755".
- Protease-sensitive_prionopathy wikiPageLength "3216".
- Protease-sensitive_prionopathy wikiPageOutDegree "25".
- Protease-sensitive_prionopathy wikiPageRevisionID "679020143".
- Protease-sensitive_prionopathy wikiPageWikiLink 14-3-3_protein.
- Protease-sensitive_prionopathy wikiPageWikiLink Alzheimers_dementia.
- Protease-sensitive_prionopathy wikiPageWikiLink Alzheimers_disease.
- Protease-sensitive_prionopathy wikiPageWikiLink Aphasia.
- Protease-sensitive_prionopathy wikiPageWikiLink Ataxia.
- Protease-sensitive_prionopathy wikiPageWikiLink Category:Transmissible_spongiform_encephalopathies.
- Protease-sensitive_prionopathy wikiPageWikiLink Cerebrospinal_fluid.
- Protease-sensitive_prionopathy wikiPageWikiLink Codon.
- Protease-sensitive_prionopathy wikiPageWikiLink Creutzfeldt-Jakob_disease.
- Protease-sensitive_prionopathy wikiPageWikiLink Creutzfeldt–Jakob_disease.
- Protease-sensitive_prionopathy wikiPageWikiLink Dementia.
- Protease-sensitive_prionopathy wikiPageWikiLink Electroencephalography.
- Protease-sensitive_prionopathy wikiPageWikiLink Genetic_code.
- Protease-sensitive_prionopathy wikiPageWikiLink Homozygous.
- Protease-sensitive_prionopathy wikiPageWikiLink Immunohistochemistry.
- Protease-sensitive_prionopathy wikiPageWikiLink Isoform.
- Protease-sensitive_prionopathy wikiPageWikiLink Magnetic_resonance_imaging.
- Protease-sensitive_prionopathy wikiPageWikiLink Mood_disorder.
- Protease-sensitive_prionopathy wikiPageWikiLink Neurodegeneration.
- Protease-sensitive_prionopathy wikiPageWikiLink Neurodegenerative_disorder.
- Protease-sensitive_prionopathy wikiPageWikiLink PRNP.
- Protease-sensitive_prionopathy wikiPageWikiLink Parkinsonism.
- Protease-sensitive_prionopathy wikiPageWikiLink Pathology.
- Protease-sensitive_prionopathy wikiPageWikiLink PrP.
- Protease-sensitive_prionopathy wikiPageWikiLink Prion.
- Protease-sensitive_prionopathy wikiPageWikiLink Protease.
- Protease-sensitive_prionopathy wikiPageWikiLink Protein.
- Protease-sensitive_prionopathy wikiPageWikiLink Protein_isoform.
- Protease-sensitive_prionopathy wikiPageWikiLink Psychosis.
- Protease-sensitive_prionopathy wikiPageWikiLink Spongiform_encephalopathy.
- Protease-sensitive_prionopathy wikiPageWikiLink Transmissible_spongiform_encephalopathy.
- Protease-sensitive_prionopathy wikiPageWikiLink Valine.
- Protease-sensitive_prionopathy wikiPageWikiLink Zygosity.
- Protease-sensitive_prionopathy hasPhotoCollection Protease-sensitive_prionopathy.
- Protease-sensitive_prionopathy wikiPageUsesTemplate Template:Merge_to.
- Protease-sensitive_prionopathy wikiPageUsesTemplate Template:Orphan.
- Protease-sensitive_prionopathy wikiPageUsesTemplate Template:Reflist.
- Protease-sensitive_prionopathy subject Category:Transmissible_spongiform_encephalopathies.
- Protease-sensitive_prionopathy hypernym Disorder.
- Protease-sensitive_prionopathy type Article.
- Protease-sensitive_prionopathy type Disease.
- Protease-sensitive_prionopathy type Article.
- Protease-sensitive_prionopathy type Disorder.
- Protease-sensitive_prionopathy comment "Protease-sensitive prionopathy (PSPr) is a neurodegenerative disorder caused by an abnormal isoform of the prion protein. Contrary to the prions in Creutzfeldt-Jakob disease (CJD), the prions in this condition are sensitive to protease activity.PSPr was first described in an abstract for a conference on prions in 2006, and this study was published in a 2008 report on 11 cases. The study was conducted by Gambetti P.".
- Protease-sensitive_prionopathy label "Protease-sensitive prionopathy".
- Protease-sensitive_prionopathy sameAs m.05b_j7f.
- Protease-sensitive_prionopathy sameAs Q7251221.
- Protease-sensitive_prionopathy sameAs Q7251221.
- Protease-sensitive_prionopathy wasDerivedFrom Protease-sensitive_prionopathy?oldid=679020143.
- Protease-sensitive_prionopathy isPrimaryTopicOf Protease-sensitive_prionopathy.