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Hematech, Inc. and Kirin Brewery Company, Ltd, announced in a report published in Nature Biotechnology, the production of healthy prion protein-knockout cows.
Prion protein is a natural cellular protein that can become misfolded into infectious particles and cause bovine spongiform encephalopathy (BSE, or "mad cow disease") and a lethal variant of Creutzfeldt-Jakob disease (vCJD) in humans. Healthy prion protein-free calves should be useful in investigating the function of normal cellular prion protein and the nature of prion diseases. Additionally, the cows should be useful as a source of prion protein-free products.
According to the Food and Drug Administration's Center for Food Safety & Nutrition, prions are associated with a group of diseases called Transmissible Spongiform Encephalopathies (TSEs). In humans, the related illness, which is suspected of being food-borne, is vCJD. The human disease, vCJD, and the cattle disease, BSE, also known as "mad cow" disease, both appear to be caused by a mutant form of prion protein. After an extended incubation period of years, these diseases result in irreversible neurodegeneration.
"By knocking out the prion protein gene and producing healthy calves, our team has successfully demonstrated that normal cellular prion protein is not necessary for the normal development and survival of cattle. The cows are now nearly two years old and are completely healthy," stated James M. Robl, Ph.D., President and Chief Scientific Officer of Hematech.
