Disease Motifs
Investigating the Biomechanics of Disease
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Prion Protein Associated Diseases

Prion diseases, which are sometimes referred to as transmissible spongiform encephalopathies (TSEs) are a collection of neurodegenerative diseases that affect humans and animals. Among the prion diseases that affect humans is Creutzfeldt-Jakob disease (CJD), which can be split in to several different categories: -

  1. Sporadic CJD, which occurs infrequently with no known cause
  2. Iatrogenic which is CJD passed on via surgery or medical procedures
  3. Familial CJD now also called genetic CJD which is the form of the disease where there is a known genetic predisposition towards being affected with CJD
  4. New variant CJD (vCJD) which has recently started to appear, mainly in the UK, but also in other countries. It has been suggested that this may well be caused as the result of eating contaminated foods such as beef from a cow with BSE.

These are really in some sense an artificial separating of the same disease in to four categories and it may well be just a pointless exercise to separate things out like this. The second group, the Iatrogenic CJD does not really concern the work here, but is only important because it is necessary to know that CJD can be spread and so can be seen as contagious. The same can be said of the fourth group which, although a serious situation if people start to become infected on a large scale, the important aspect of this is that there seems to be a genetic factor that increases the susceptibility of some of the victims. So perhaps the real division of CJD should be: sporadic, CJD of no known cause; acquired, by contaminated surgical equipment or eating infected material; or genetic susceptibility.But even this grouping can be a bit deceptive when one considers there might be some genetic bias in sporadic CJD, and presumably those who have a genetic susceptibility in familial CJD maybe only more prone to developing a disease if they are expose to the same environmental factor (if one exists) that the people with sporadic CJD have been exposed to.

Other human prion diseases include:-

  1. Kuru, which as mentioned above is a disease that was found to be spread by cannibalism
  2. Gerstmann-Straussler-Scheinker syndrome (GSS or sometimes GSD)
  3. Fatal Familial Insomnia (FFI).
Some of the major prion diseases that appear in animals are:-
  1. Bovine Spongiform Encephalopathy (BSE) which appeared in cattle in the UK in the eighties
  2. Chronic Wasting Disease (CWD) in deer
  3. Scrapie in sheep.

This whole Prion Section will be gradually built up to include more information and this will be an ongoing situation. It may be necessary to rework articles that have already been uploaded to the site as I have decided that I may not be happy with a particular part of what I have previously written. If you notice any mistakes whether these are simple typos or even incorrect information please feel free to email me and tell me, as I would greatly appreciate it.


Some links to prion protein analysis

PRIO_HUMAN protein sequence analysis

PRIO_HUMAN polymorphic sequence analysis