Publication: Current concepts in human prion protein (Prp) misfolding, Prnp gene polymorphisms and their contribution to Creutzfeldt-Jakob Disease (CJD)
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Date
2007
Authors
Michalczyk, K. ; Ziman, M.
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Publisher
Murcia : F. Hernández
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DOI
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info:eu-repo/semantics/article
Description
Abstract
Transmissible spongiform encephalopathies
are a group of neural degenerative diseases that may be
infectious, sporadic, or hereditary and are associated
with an abnormally folded prion protein. Unfortunately
at the current time it is not at all clear what the normal
structure of the prion protein actually is or how it is toxic
to cells.
Extensive research on prion diseases has led to a
dramatic increase in understanding of the pathogenesis
of prion disorders, which will hopefully lead to the
development of effective treatments. The inability to
detect the disease in blood using current technology has
made screening difficult. While fortunately there has
been a decline in the number of clinical cases of
transmissible variant CJD, evidence indicates that very
long incubation periods can occur in humans so there
may be a long slow, gradual epidemic. In particular,
clinical cases in genotypes other than those homozygous
for methionine at codon 129 of PRNP have not yet
occurred, but such cases might be expected to have
longer incubation periods and show differences in
pathology to those seen to date.
proportion of infected animals develop sub-clinical
disease. Moreover, results from a large prevalence study
in humans show that several cases test positive but do
not develop clinical disease. It is possible therefore that
further cases of secondary transmission could occur by
iatrogenic spread, which could result in vCJD persisting
in the UK at low levels for many years, highlighting the
importance of continued vigilance.
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