Prions are responsible for «mad cow» disease in cattle,
scrapie in sheep, and Creutzfeld - Jakob and other fatal ailments in humans.
Scientists have located two possible receptors for the so - called prion protein (PrP) believed to be at fault in fatal neurological conditions such as «mad cow disease,» Creutzfeldt - Jakob disease in humans, and
scrapie in sheep.
This accomplice, the researchers say, allows the protein to cause disorders like «mad cow» disease in cattle,
scrapie in sheep, and Creutzfeldt - Jakob disease (CJD) in humans.
Other prion diseases include
scrapie in sheep, chronic wasting disease in deer, elk and moose, and bovine spongiform encephalopathy in cattle.
Scientists studying the commonest of the diseases in the group,
scrapie in sheep, concluded decades ago that no virus or bacterium could be involved because infectious material appeared to contain no DNA.
Other prion diseases include
scrapie in sheep; chronic wasting disease in deer, elk and moose; and bovine spongiform encephalopathy (BSE), or mad cow disease, in cattle.
They found that although the protein stayed soluble for a week or two, it eventually polymerized into long fibers resembling those in so - called prion diseases — brain diseases such as
scrapie in sheep, «mad cow disease» in cattle, and Creutzfeldt - Jakob disease in humans.
Several fatal neurological diseases — including Creutzfeldt - Jakob disease (CJD) in humans and
scrapie in sheep — are marked by the accumulation of protein deposits in the brain.
Degenerative brain diseases like mad cow disease (officially known as bovine spongiform encephalopathy, or BSE),
scrapie in sheep, and vCJD in humans are thought to be caused by prions, misfolded versions of a normal cellular protein called PrPC.
Not exact matches
In addition to chronic wasting disease, examples include scrapie and bovine spongiform encephalopathy (or «mad cow disease») in animals and variant Creutzfeldt - Jakob disease in human
In addition to chronic wasting disease, examples include
scrapie and bovine spongiform encephalopathy (or «mad cow disease»)
in animals and variant Creutzfeldt - Jakob disease in human
in animals and variant Creutzfeldt - Jakob disease
in human
in humans.
The duo admits they have little evidence to back up their proposal, but they point to studies
in their lab on mice, which were altered to be susceptible to both BSE and
scrapie prion infection.
Because the
scrapie strain propagates faster, Prusiner says, it would vastly outnumber the BSE / nvCJD strain
in infected sheep.
Prion specialist Moira Bruce of the Institute for Animal Health's Neuropathogenesis Unit
in Edinburgh says that sheep experimentally infected with BSE become ill about as fast as sheep naturally infected with
scrapie.
The heat rendering process, they suggest, may
in fact destroy or otherwise inactivate the
scrapie prions, paving the way for the scarcer but more hardy BSE prions to infect the cattle.
Scientists generally assume that BSE arose
in cattle whose feed was enriched with a high - protein supplement: sheep parts, unfortunately infected with
scrapie.
At GTC, the
scrapie - free goats brought
in from New Zealand are penned within a 190 - acre enclosure on a 300 - acre plot
in Charlton, Mass..
In the US, consumer rights groups won a ban on the purchase of meat from
scrapie flocks because no one could rule out absolutely the possibility of transmission to humans.
According to Richard Kimberlin, of the AFRC / MRC Neuropathogenesis Unit
in Edinburgh: «The similarities are enough to make us think that it's
in the
scrapie family, but without evidence of transmission it's impossible to say anything more certain».
This turned out to be a normal protein
in the cells of organisms throughout the animal kingdom — but
in brains infected with
scrapie and related diseases it turns up
in both a normal, soluble form and an abnormal, insoluble form which accumulates
in deposits that eventually kill the cells.
In their study, the NIAID scientists injected infectious
scrapie prion protein into the brains of mice.
The decrease prompts hopes that, unlike
scrapie, the spongiform encephalopathy
in sheep, BSE will not gain a permanent foothold
in Britain.
Although endemic
in Europe for centuries, the sheep disease known as
scrapie achieved notoriety only during the 1980s, when it was apparently transmitted to cows
in Britain via infected sheep remains
in cattle feed, thereby causing mad cow disease.
It cause animal brains to turn into a spongy mess
in scrapie, a disease of sheep, and
in bovine spongiform encephalopathy (BSE or «mad cow disease»), as well as
in human prion diseases such as CJD.
Scientists now know that
scrapie is caused by misfolding proteins
in the brain called prions, the same mechanism that causes Creutzfeldt - Jakob disease
in humans.
The transmission of different
scrapie prions
in these mice also led to the propagation of prions that appear identical to those causing sCJD
in humans.
The new finding offers direct, physical evidence supporting protein - based inheritance, thus strengthening the «prion hypothesis» of the cause of neurodegenerative diseases
in mammals, such as sheep
scrapie, mad cow disease (or bovine spongiform encephalopathy) and the kuru disease of the Papua New Guinea tribes.
BSE (mad cow disease),
scrapie and other known TSE's, do not occur
in New Zealand.
And while kuru is of course a singular condition, its cousins — including mad cow and
scrapie — are the stuff of everyday Western life, and it was for this discovery that he was awarded the Nobel Prize
in Physiology or Medicine
in 1976.
Dr. Cezar started her career at APHIS serving as a Veterinary Medical Officer, a role under Veterinary Services, where she worked firsthand
in surveillance of critical public health diseases such as Chronic Wasting Disease and
Scrapie.
BSE /» Mad cow disease» and
scrapie happened
in the UK long years before it was an issue
in the USA.