Not exact matches
Using a mouse model that expresses an altered form
of the
normal human
prion protein, researchers at University
of California, San Diego School
of Medicine have determined why the human
proteins aren't corrupted when exposed to the elk
prions.
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.
He and colleagues at the University
of California, San Francisco, injected the brains
of mice with
prions they had created in the lab by misfolding
normal prion protein, known as PrP.
Misshapen
proteins called
prions lie at the root
of mad cow disease and similar brain ailments, but the role
of these molecules in their
normal form remains unclear.
The brains
of mice genetically modified to lack
normal prion proteins had significantly higher beta - amyloid levels.
In an article entitled «
Prion protein modulates monoaminergic systems and depressive - like behavior in mice» and published in the Journal of Biological Chemistry, the group shows that mice lacking normal prions show a depressive - like behavior similar to depression symptoms found in patients with Alzheimer's and prion diseases, namely Creutzfeldt - Jakob Disease (CJD), variant Creutzfeldt - Jakob Disease (vCJD), Gerstmann - Sträussler - Scheinker syndrome, Fatal Familial Insomnia and
Prion protein modulates monoaminergic systems and depressive - like behavior in mice» and published in the Journal
of Biological Chemistry, the group shows that mice lacking
normal prions show a depressive - like behavior similar to depression symptoms found in patients with Alzheimer's and
prion diseases, namely Creutzfeldt - Jakob Disease (CJD), variant Creutzfeldt - Jakob Disease (vCJD), Gerstmann - Sträussler - Scheinker syndrome, Fatal Familial Insomnia and
prion diseases, namely Creutzfeldt - Jakob Disease (CJD), variant Creutzfeldt - Jakob Disease (vCJD), Gerstmann - Sträussler - Scheinker syndrome, Fatal Familial Insomnia and kuru.
«Our study sounds a serious warning about the possible detrimental side effects
of this strategy, since we have shown that such antibodies cause dramatic degeneration
of nerve cells by interfering with the
normal on - off function
of the
prion protein,» he added.
A
prion version
of a
protein can perpetuate itself in an infectious manner by converting
normal forms
of that
protein into the
prion version.
The researchers also found that although the
normal version
of Rho suppressed gene activity in E. coli, many genes were active when the
protein was in its
prion form.
Yeast can also carry
prions, and the duo fused part
of a yeast
prion protein called Sup35 to a
normal rat
protein that controls the transcription
of DNA into RNA.
Worse, instead
of being destroyed and recycled by the cell — the typical fate
of misfolded
proteins —
prions resist degradation and cause other
normal prion proteins to misfold.
Finally, he looked to see whether the
prion state
of Rnq1 could entice Rnq1
proteins in the
normal state to switch to the
prion state.