Linked Life Data

1676992

Source:http://linkedlifedata.com/resource/pubmed/id/1676992

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
1991-8-20
pubmed:abstractText
The spongiform encephalopathies belong to the group of "slow virus infections" of the nervous system, characterized by a long incubation period, a protracted course and involvement of the nervous system with a lethal outcome. In contrast to the conventional virus infections, such as visna in sheep and progressive multifocal leukoencephalopathy (PML) in humans, the etiological agent for the spongiform encephalopathies has not been clearly defined. The known forms in animals are scrapie in sheep and goats, transmissible mink encephalopathy, and chronic wasting disease of mule deer and elk. In humans, the three known forms are Kuru, now mainly of historical interest, Creutzfeldt-Jakob (CJ) disease and the syndrome of Gerstmann-Straussler-Scheinker (GSS). An important feature of these diseases is the lack of an immune response by the host, which is reflected in the absence of inflammatory infiltrates in the affected tissues. In this editorial the two most important hypotheses on the etiology and pathogenesis of this group of conditions will be discussed. The "prion" hypothesis considers the possibility that a protein, derived from a normal component of the neuronal membranes may have a leading role, not only in the infectivity and transmissibility of these diseases, but in the pathological changes that ensue. A single host gene would code for both the normal and altered proteins. The altered protein would be partially insoluble and would result in the deposition of fibrils and rods which would precipitate in the form of amyloid. Since the involved protein would be coded for by the host, there would be no immune response against it.(ABSTRACT TRUNCATED AT 250 WORDS)
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Apr
pubmed:issn
0392-0461
pubmed:author
pubmed:issnType
Print
pubmed:volume
12
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
147-53
pubmed:dateRevised
2004-11-17
pubmed:meshHeading
pubmed:year
1991
pubmed:articleTitle
Human and experimental spongiform encephalopathies: recent progress in pathogenesis.
pubmed:publicationType
Editorial