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.
The authors show that a panel of sheep
scrapie prions transmit to several of these mouse models with efficiency comparable to that of cattle BSE.
Moreover, mice infected
with scrapie progressed more quickly to disease.
Other prion diseases
include scrapie in sheep; chronic wasting disease in deer, elk and moose; and bovine spongiform encephalopathy, or mad cow disease, in cattle.
The potential
of scrapie prions to infect humans was unknown.
Ma wholeheartedly rejects this possibility: «There's no way» the mice could have contracted a natural prion disease
like scrapie, he says, because his lab has not worked with naturally occurring prions.
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.
If sheep do indeed catch
scrapie by eating mite - infested hay, Wisniewski says, there may be a simple way to control the disease.
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.
Extrapolating from these findings, says Prusiner, «there seems to be a reservoir of different [prion] strains in sheep»: a fast - acting one that
causes scrapie and a slower one that causes BSE / nvCJD.
You wipe out the
labile scrapie prion» by heat rendering, Prusiner says.
While the diseases have similar symptoms, BSE prions do not
trigger scrapie and vice versa.
She wonders if the animals actually
contracted scrapie, another prion disease, because of lab contamination.
Many countries ban the import of sheep from areas
where scrapie occurs.
They found that the transgenic mice were susceptible to classical and atypical Bovine Spongiform Encephalopathy prions, and also to mouse -
derived Scrapie prions.
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.
But while the source of mad cow disease is fairly well established, no one knows
how scrapie infects sheep.
After about a year had passed, 14 of the mice
developed scrapie's symptoms: a wobbly walk, difficulty grasping, and a rigid tail.
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.
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.
It's almost impossible to get your hands on brain here because of the mad cow /
scrapie scare.
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.
I think that recycling is a great thing but this sounds more
like scrapiing the bottom of the barrell for scraps..
By this time it was known that BSE was a prion disease but whether the infective prion came
from scrapie - infected sheep or another source is still not known.
This group of progressive diseases
includes scrapie in sheep and goats, chronic wasting disease in mule deer and transmissible mink encephalopathy.
Resistance of cattle to
scrapie by the oral route.
Other prion diseases include
scrapie in sheep, chronic wasting disease in deer, elk and moose, and bovine spongiform encephalopathy in cattle.
When these animals were given low doses
of scrapie, disease onset was delayed or in some cases even prevented, although large doses of prion could override the effect.
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.
Stanley Prusiner of the University of California, San Francisco (UCSF), suggested that the aberrant prion proteins thought to cause bovine spongiform encephalopathy (BSE), as the disease is formally known, may coexist in sheep along with the prions implicated in
scrapie, an illness that does not appear to infect humans.
They speculate that sheep may be infected both with BSE and
scrapie prions, but only the latter cause disease in sheep.
The leading theory is that a technique for heat rendering of sheep parts that became mandatory in the 1970s somehow altered
the scrapie prions enough to allow them to jump the species barrier and cause BSE.
The idea is that
scrapie prions might somehow interfere with the infectivity of BSE prions.
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 humans.
Because
the scrapie strain propagates faster, Prusiner says, it would vastly outnumber the BSE / nvCJD strain in infected sheep.
With the scale of the nvCJD threat to public health still unclear, scientists have been busy investigating the relationship between the strains of prions blamed for neurodegenerative illnesses, including the presumed connection between BSE and
scrapie.
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.
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.
«
Scrapie prions may even protect humans [from BSE].»
But, making assumptions based on past experience with
scrapie, the Southwood committee decided that it was unlikely that BSE could be passed to humans.
Scientists generally assume that BSE arose in cattle whose feed was enriched with a high - protein supplement: sheep parts, unfortunately infected with
scrapie.
PrP is expressed at high levels in the brain, and prion diseases, including Creutzfeldt - Jakob disease (CJD) in humans, bovine spongiform encephalopathy (BSE, or «mad cow disease») in cows, and
scrapie in sheep, wreak havoc on the brain and other neural tissues.
The scrapie agent causes a degenerative nervous system disease in sheep and goats.
Presumably, nucleophilic residues within
a scrapie agent protein undergo carbethoxylation on reaction with diethyl pyrocarbonate, and subsequent addition of hydroxylamine displaces these carbethoxy groups.
Chemical modification of
the scrapie agent by diethyl pyrocarbonate reduced the titer 1000-fold.
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..
Like
scrapie and the other diseases, bovine spongiform encephalopathy is insidious and progressive.
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.