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The Human
Prions
Kuru the “Laughing Death” was first observed in the Fore
tribe of New Guinea where a cannibalistic ritual that involved consumption of the
brains of the dead, a practice believed to honor their elders. This
practice is now believed to have stopped.
CJD or the Cruetzfeldt – Jakob disease is believed to possess an
iatrogenic route of transmission too, which has occurred through the use of
contaminated syringes and other medical equipment. Injections of the human growth
hormone from the pituitary before the recombinant hormone was available
also led to the propagation of the disease.
Gertsmann – Straussler – Scheinker and Fatal Familial Insomnia involve point mutations in the human Prion gene.
Alpers syndrome is a recent prion disease that has been found to occur in
infants.
The Animal Prions
Scrapie is one of the well known animal Prion
diseases, found in goats and sheep. It results in symptoms of loss of
coordination, incapacitation and itching.
The itch causes the animal to
scrape itself against a hard surface causing a loss of hair or wool,
leading to the name Scrapie
Mad Cow Disease or the Bovine
Spongiform Encephalopathy has received a lot of attention. It was
first identified in 1986 by George A.H.Wells and
John W. Wilesmith in Weybridge, England in cows
that were uncoordinated and unusually apprehensive. The source of the
emerging epidemic was soon traced to a food supplement that included meat
and bone meal from dead sheep!!!!!!
It is believed that people who
might consume tainted meat from such sources may have been infected with a
variant form of CJD known as vCJD.
Some
other Prion diseases found in animals are the Transmissible Mink
Encephalopathy, The Chronic Wasting disease of deer and elk, and the Feline
Spongiform Encephalopathy. All of which demonstrate similar symptoms of
loss of coordination attributed to neuronal damage.
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