Protease resistant prion proteins are not present in sporadic "poor outcome" schizophrenia.

نویسندگان

  • S E Arnold
  • J Q Trojanowski
  • P Parchi
چکیده

Various clinical and epidemiological data have suggested the possibility of infectious mechanisms in schizophrenia. In addition, lengthy prodromal psychiatric symptoms can presage the development of Creutzfeldt-Jakob disease, a prototypical prion disorder. Accordingly, the presence of human protease resistant prion proteins (PrPres) was assessed in postmortem frontal cortical and thalamic tissues from a prospectively accrued and well characterised sample of elderly patients with chronic, sporadic, "poor outcome" schizophrenia using a sensitive immunoblot assay. No PrPres was found in the brains of any of the cases, providing evidence against a role for abnormal prion proteins in the pathogenesis of schizophrenia.

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SHORT REPORT Protease resistant prion proteins are not present in sporadic “poor outcome” schizophrenia

Various clinical and epidemiological data have suggested the possibility of infectious mechanisms in schizophrenia. In addition, lengthy prodromal psychiatric symptoms can presage the development of Creutzfeldt-Jakob disease, a prototypical prion disorder. Accordingly, the presence of human protease resistant prion proteins (PrP) was assessed in postmortem frontal cortical and thalamic tissues ...

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Pathologic prion protein spreading in the peripheral nervous system of a patient with sporadic Creutzfeldt-Jakob disease.

BACKGROUND Involvement of the peripheral nervous system in the pathogenesis of prion diseases is becoming increasingly evident. However, pathologic protease-resistant prion protein deposition in the peripheral nerves of patients with Creutzfeldt-Jakob disease has never been demonstrated, to our knowledge. OBJECTIVE To determine whether mutated prion protein accumulation could be shown in the ...

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Coexistence of protease sensitive and resistant prion protein in 129VV homozygous sporadic Creutzfeldt–Jakob disease: a case report

UNLABELLED INTRODUCTION The coexistence of different molecular types of classical protease-resistant prion protein in the same individual have been described, however, the simultaneous finding of these with the recently described protease-sensitive variant or variably protease-sensitive prionopathy has, to the best of our knowledge, not yet been reported. CASE PRESENTATION A 74-year-old Ca...

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The prion protein protease sensitivity, stability and seeding activity in variably protease sensitive prionopathy brain tissue suggests molecular overlaps with sporadic Creutzfeldt-Jakob disease

INTRODUCTION Variably protease sensitive prionopathy (VPSPr) is a recently described, sporadic human prion disease that is pathologically and biochemically distinct from the currently recognised sporadic Creutzfeldt-Jakob disease (sCJD) subtypes. The defining biochemical features of the abnormal form of the prion protein (PrPSc) in VPSPr are increased sensitivity to proteolysis and the presence...

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Distinct glycoform ratios of protease resistant prion protein associated with PRNP point mutations.

Inherited prion diseases are neurodegenerative disorders caused by autosomal dominant mutations in the human prion protein gene (PRNP). Kindred with inherited prion disease can show remarkable phenotypic variability that has yet to be explained. Here we report analysis of protease resistant disease-related prion protein (PrP(Sc)) isoforms from a range of inherited prion disease cases (point mut...

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عنوان ژورنال:
  • Journal of neurology, neurosurgery, and psychiatry

دوره 66 1  شماره 

صفحات  -

تاریخ انتشار 1999