Novel prion protein conformation and glycotype in Creutzfeldt-Jakob disease.
Arch Neurol
; 64(4): 595-9, 2007 Apr.
Article
en En
| MEDLINE
| ID: mdl-17420324
ABSTRACT
OBJECTIVE:
To describe a novel molecular and pathological phenotype of Creutzfeldt-Jakob disease. Patient A 69-year-old woman with behavioral and personality changes followed by rapidly evolving dementia.RESULTS:
Postmortem examination of the brain showed intracellular prion protein deposition and axonal swellings filled with amyloid fibrils. Biochemical analysis of the pathological prion protein disclosed a previously unrecognized PrP(Sc) tertiary structure lacking diglycosylated species. Genetic analysis revealed a wild-type prion protein gene. The prion agent responsible for this atypical phenotype was successfully passaged to bank voles.CONCLUSION:
To our knowledge, our results define a new human prion disorder characterized by intracellular accumulation of a novel type of pathological prion protein.
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Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Síndrome de Creutzfeldt-Jakob
/
Proteínas PrPSc
Límite:
Aged
/
Animals
/
Female
/
Humans
Idioma:
En
Revista:
Arch Neurol
Año:
2007
Tipo del documento:
Article
País de afiliación:
Italia