On a related topic, from the autopsy report released on Fernando Valenzuela the former Dodger great, it seems he suffered from CJD, the human version of chronic wasting disease. Here is some interesting information as to how people can become infected without eating infected meat.
The normal, harmless prion protein is usually designated as PrPC ("C" stands for cellular) and the abnormal, infectious form (which causes the disease) is PrPSc ("Sc" stands for prototypical prion disease–scrapie).
In the acquired form of the disease, the infectious PrPSc comes from the outside the body, for example, through contaminated meat as is seen in variant CJD.
In the hereditary form, infectious prions can arise when a mutation occurs in the gene for the body's normal prion protein. As the mutated PrPC duplicates itself, it spontaneously changes shape into the infectious form. (Prions themselves do not contain genetic information and do not require genes to reproduce themselves.) If the prion protein gene is changed in a person's sperm or egg cells, the changed gene can be transmitted to the person's children. Specific changes found in each family affects how often the disease appears and what symptoms are most noticeable. However, not all people with mutations in the prion protein gene develop CJD.
In the sporadic form, the infectious prions are believed to be made by an error in the cell's machinery that makes proteins and controls their quality. These errors are more likely to occur with aging.
|