Stephen J. Schueler, M.D.

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Creutzfeldt-Jakob Disease Variant Underlying Cause

Variant Creutzfeldt-Jakob disease is caused by a tiny protein, called a prion. The prion is present in cattle that have bovine spongiform encephalopathy (BSE). Humans are thought to become infected by eating cattle that are infected with the prion.

Prion proteins occur in both a normal form, which is a harmless protein found in the body's cells, and in an infectious form, which causes Variant Creutzfeldt-Jakob disease. The infectious form of the protein has a different folded shape than the normal form. Once the abnormal prions appear, they clump together within cells and somehow trigger cell death.

Continue to Creutzfeldt-Jakob Disease Variant Transmission

Last Updated: Jul 15, 2009 References
Authors: Stephen J. Schueler, MD; John H. Beckett, MD; D. Scott Gettings, MD
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PubMed Creutzfeldt-Jakob Disease Variant References
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  2. Brown P: The risk of bovine spongiform encephalopathy ('mad cow disease') to human health. JAMA 1997 Sep 24; 278(12): 1008-11. [9307349]
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  4. Roma AA, Prayson RA. Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: how safe is eating beef? Cleve Clin J Med. 2005 Mar;72(3):185-6, 189-90, 192-4. [15825799]
  5. Weihl CC, Roos RP. Creutzfeldt-Jakob disease, new variant creutzfeldt-jakob disease, and bovine spongiform encephalopathy. Neurol Clin. 1999 Nov;17(4):835-59. [10517931]
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