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Prions in
man - Prions were associated with the Kuru disease of head-hunting cannibalistic tribes in New Guinea (with brain to finger then mouth or skin wound transfer). - Familial Creutzfeldt-Jakob disease (CJD) is caused by spontaneous mutations of PrPc which is high in Libyan Jews in Israel where this mutation was discovered. - Until BSE other causes of CJD are sporadic although iatrogenic transmission via pituitary hormone or brain surgery occurred. - Variant CJD is now considered to be identical to BSE and, like BSE, is foodborne. |