Document Detail


Management and prevention of human prion diseases.
MedLine Citation:
PMID:  19818228     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Prion diseases are a group of fatal neurologic disorders that affect humans and animals and for which there is no available therapy. The basic pathogenic mechanism is linked to posttranslational changes of the host cellular prion protein (PrP(c)) into a pathologic conformer (PrP(TSE)) that has a strong tendency to aggregate and form amyloid fibrils. In humans, the most common form of the disease is sporadic Creutzfeldt-Jakob disease (CJD), which equally affects females and males of all ages and all ethnic groups. Sporadic CJD has an overall mortality rate of approximately one to two cases per million people per year, with peak incidence in individuals 60 to 70 years old. Approximately 10% to 20% of CJD cases appear within families and are linked to point or insert mutations in the prion protein gene (PRNP). Both sporadic and genetic prion disorders are transmissible to a wide range of laboratory animals by the injection of crude brain homogenates.
Authors:
Silvia Graziano; Maurizio Pocchiari
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Current neurology and neuroscience reports     Volume:  9     ISSN:  1534-6293     ISO Abbreviation:  Curr Neurol Neurosci Rep     Publication Date:  2009 Nov 
Date Detail:
Created Date:  2009-10-12     Completed Date:  2009-12-15     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100931790     Medline TA:  Curr Neurol Neurosci Rep     Country:  United States    
Other Details:
Languages:  eng     Pagination:  423-9     Citation Subset:  IM    
Affiliation:
Department of Cell Biology and Neurosciences, Istituto Superiore di Sanità, Rome, Italy. maurizio.pocchiari@iss.it
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MeSH Terms
Descriptor/Qualifier:
Blood Transfusion / adverse effects
Family Health
Female
Genetic Predisposition to Disease
Humans
Male
Prion Diseases / diagnosis*,  etiology,  genetics,  prevention & control*
Prions / genetics
Risk Factors
Urine
Chemical
Reg. No./Substance:
0/PRNP protein, human; 0/Prions

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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