Define concerns of food safety-Prions

Bovine  Spongiform Encephalopathy (BSE)  and Creutzfeldt-Jakob Disease  (CJD) have  been  the highly publicized food safety hazard. You may  recall reading about  the mad-cow disease and large number of diseased cattle being destroyed in  the UK,  or about the ban on British beef worldwide. Why was  this action being taken? What was  the hazard? The hazard was from  the Prions (pronounced pree- ahns). Prions entered the public's consciousness during the mad cow epidemic that hit England  in 1986.

What are Prions?

Prions,  the cause of BSE and  CJD, are an entirely new source of food  borne diseases.  Prions, is the abbreviation  of proteinaceous particles. The  word  'Prion'  was coined  by  Dr:  Stanley Prusiner  to indicate that this disease was caused by  a "proteinaceous infectious agent." Prions,

In  fact, are modified forms of  a  normal  protein called  as PrPC.The protein  that causes this  and  all other  prion  diseases  is  called  PrPSC,  which  stands  for prion protein of scrapie.  Prions enter cells and apparently convert normal proteins found within the cells into prions  just like themselves. The proteins accumulate  in the brain causing holes or plaques and the subsequent clinical symptom leading to death. The medical term for  the prion  diseases  is  "spongiform encephalopathies,"  in acknowledgement that the sick brains are riddled with holes and have taken the form of  sponges.

 Transmissible spongiform encephalopathy in animals and humans  are caused  by  prions. Prions, therefore,  can  be infectious and can  cause  infectious diseases. The second and potentially more troubling aspect is that, like other infectious agents, prions can jump  species'  barriers and cause d&dly  diseases in humans. Only time will tell  how big  a problem the prions will be both  as  the agents  of dreadful dlseases of the human nervous system and as vectors of diseases from other species.

BSE was first confirmed  in cattle in 1986 in UK. It  is commonly accepted that BSE was  first caused in Britain when cattle were fed carcass meal from scrapie infected sheep. Since 1999,  other countries in Europe e.g., Belgium, Denmark, France, Germany, Ireland,  Italy, Netherland,  Portugal etc.  have  reported confirmed  cases  of  BSE.

Humans contracted the non-classic form  of  CJD,  called new variant  CJD  (vCJD) after  consuming cattle meat, in particular, the nervous tissue.