Creutzfeldt-Jacob Disease (CJD)
Creutzfeldt-Jacob Disease (CJD) is one of a group of diseases called spongiform encephalopathies. (Spongiform because the affected brain resembles a sponge, with lots of cystic spaces, while encephalopathy simply means a problem causing structural change in the brain). These include BSE in cattle and Scrapie in sheep. There is rapidly progressive degeneration of brain tissue. In the human this causes dementia and jerking of the limbs. The cause is thought to be an abnormal protein called a prion. Our cells contain a protein called PrP. Prions are made as a result of a mutation in the gene that codes for this protein. They are thought to be able to reproduce themselves and are incorporated into cell walls, where they cause the cell to degenerate.
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