Linked Life Data

12505278

Source:http://linkedlifedata.com/resource/pubmed/id/12505278

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:dateCreated
2002-12-30
pubmed:abstractText
One of the most remarkable changes in medicine during the last 20 years of the 20th century was the shift from the clinical-neuropathological classification of Creutzfeldt-Jakob disease (CJD) and related disorders as 'transmissible spongiform encephalopathies' to a molecular-etiologic classification as 'prion diseases'. We now know that these diseases are caused by abnormalities of the prion protein (PrP). Specifically, CJD is caused by the conversion of the normal, protease-sensitive PrP isoform, designated PrP(C), to a protease resistant isoform, designated PrP(Sc). PrP(Sc) forms into an infectious particle, named a 'prion', that can transmit the disease. Accumulation of PrP(Sc) in the brain causes neurodegeneration. The main goals of this review are to summarize our understanding of the attributes of the PrP molecule that give it the properties of an infectious agent and to describe how different alterations of the PrP molecule cause the multiple known prion disease variants. Finally, the emergence of a new variant of CJD in Great Britain and to a lesser extent in Europe and its relationship to the emergence of a particularly virulent form of bovine spongiform encephalopathy will be discussed.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Dec
pubmed:issn
0300-483X
pubmed:author
pubmed:issnType
Print
pubmed:day
27
pubmed:volume
181-182
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
9-16
pubmed:dateRevised
2007-11-14
pubmed:meshHeading
pubmed:year
2002
pubmed:articleTitle
Fundamentals of prion biology and diseases.
pubmed:affiliation
Department of Pathology (Neuropathology), HSW 430, University of California, San Francisco, CA 94143-0506, USA. sdearmo@itsa.ucsf.edu
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S., Review, Research Support, Non-U.S. Gov't