Main name of condition: Prion diseases
Other names or spellings: Transmissible Spongiform Encephalopathy, Transmissible Spongiform Encephalopathies, TSE
Introduction
Transmissible spongiform enchephalalopathies or "prion diseases" such as Creuzfeldt-Jakob Disease in man and Bovine Spongiform Encephalopathy in cattle are attracting much media interest due to the possible link between BSE and a novel form of CJD "vCJD".
These diseases are thought to be caused by unusual infectious agents called prions. These are abnormal forms of a normal protein found in the membranes of our cells, especially those in the brain and spinal cord, and in some lymphatic tissues.
The physiological role of the constitutive form, PrPC, remains controversial in spite of its evolutionary conservation. The first reports on Prnp0/0 mice (prion knock out mice), which do not make prion protein, identified no behavioural or physiological changes associated with loss of PrP(Büeler,1992) but more recent reports revealed abnormalities in circadian rhythms and sleep[Tobler,1990).
We work on the physiological role of prion protein - by looking at neuronal properties of brain slices lacking PrP and conclude that several properties change in the hippocampus.(Huber,1999)
We also work on the pathophysiology of diseases such as scrapie. with a view to understanding how this kind of disease disrupts brain function, and perhaps how it gets progressively worse. In scrapie we have found that brain function changes before the classical spongy histology develops. This may correspond to some human cases of prion disease which lack the classical histopathology. We now have evidence that, late in the progress of the disease, the properties of individual neurons changes radically.
Cause details for Prion diseases:
Research suggests that TSEs are caused by an abnormal version of a protein called a prion (prion is short for proteinaceous infectious particle).......
