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In 1907, Dr. Alzheimer published a treatise about a disease that would one day carry his name. He had two young colleagues who worked with him, Dr. Creutzfeldt and Dr. Jakob, and they too identified a similar brain-wasting disease that now has Europe in a panic. The brains of cows turn into a sponge-like mass and their behavior is called "mad." The human variant of Mad Cow Disease has been named Creutzfeldt Jakob Disease, or CJD. The protein causing CJD has no DNA, and has been described as more like a crystal than cellular material. In labs, 1000 degree Fahrenheit heat does not destroy this protein particle. Some scientists say that once infected, the incubation period can last anywhere from one month to thirty years. As the human brain turns into a sponge, this spongioform encephalitic condition physically debilitates those so infected.

Prion Diseases This document describes infectious agents which (almost certainly) do not have a nucleic acid genome. It seems that a protein alone is the infectious agent. The infectious agent has been called a prion. A prion has been defined as "small proteinaceous infectious particles which resist inactivation by procedures that modify nucleic acids". The discovery that proteins alone can transmit an infectious disease has come as a considerable surprise to the scientific community. Prion diseases are often called spongiform encephalopathies because of the post mortem appearance of the brain with large vacuoles in the cortex and cerebellum. Probably most mammalian species develop these diseases. Latest: BSE in the USA

Mad Cow Disease Images & BSE pictures. Illustration of prion replication and spread at the cellular level. Prions are implicated in BSE (Bovine Spongiform Encephalopathy or Mad Cow Disease) and its human counterpart nvCJD (new variant Creutzfeldt Jakob Disease). These and similar diseases are known as TSEs (Transmissible Spongiform Encephalopathies). TSEs afflict other species, most famously sheep (scrapie).

Official Mad Cow Disease Home Page 7,651+ articles on mad cow and new variant Creutzfeldt-Jakob disease, prions, bovine spongiform encephalopathy, scrapie, BSE, CJD, CWD, TME, and TSE. Last Updated: 17 Apr 01 . . a project of the Sperling Biomedical Foundation .

The National Prion Disease Pathology Surveillance Center was established at the Division of Neuropathology of Case Western Reserve University in September 1997. Several European countries have also established surveillance centers to monitor the occurrence of prion diseases or spongiform encephalopathies, in response to the epidemic of Bovine Spongiform Encephalopathy (BSE) or “mad cow disease” that occurred in the United Kingdom during the 1980’s.

"A Look Inside a Rendering Plant" (by GS): "Rendering has been called "the silent industry." Each year in the US, 286 rendering plants quietly dispose of more than 12.5 million tons of dead animals, fat and meat wastes. As the public relations watchdog newsletter PR Watch observes, renderers "are thankful that most people remain blissfully unaware of their existence."

"Food not Fit for a Pet" (by Dr. Wendell O. Belfield): "Some of these dead pets -- those euthanized by veterinarians -- already contain pentobarbital before treatment with the denaturing process. According to University of Minnesota researchers, the sodium pentobarbital used to euthanize pets "survives rendering without undergoing degradation." [Short, but powerful article by an expert]

"Mad cow outbreak may have been caused by animal rendering plants" (NY Times News Service): "Renderers in the United States pick up 100 million pounds of waste material every day -- a witch's brew of feet, heads, stomachs, intestines, hooves, spinal cords, tails, grease, feathers and bones. Half of every butchered cow and a third of every pig is not consumed by humans. An estimated six million to seven million dogs and cats are killed in animal shelters each year, said Jeff Frace, a spokesman for the American Society for the Prevention of Cruelty to Animals in New York City."

"The Rendering Industry: Big Business in By-Products" (by Kieran Mulvaney): "Processed cow fats are sometimes used to make cookies and salty snacks taste rich and to make lipsticks glide smoothly. Cow proteins show up in shampoo. Collagen, extracted from the inner layer of cattle hide, is used to balm wounds and cosmetically puff up lips. Gelatin, refined from cattle hide and bones, is found in such foods as ice cream, gummy candies and marshmallows--as well as the capsules encasing drugs."

"How Dead Pets, Bad Brains, and Free Speech Landed Me in Amarillo" (by Van Smith): "We were at once aghast, amused, and skeptical. "No, really, it's true," they said blandly, sensing our doubts. "We pick up dead pets from the SPCA and take them to the plant. The plant cooks up the carcasses and other things to make stuff that goes into pet food. Honest."

FAO Standard for Fats and Oils from Animal Source

Meat groups protest proposed FDA restrictions on animal feed A coalition of agricultural organizations led by the American Meat Institute (AMI) is arguing that no scientific reason exists for FDA's recent proposed changes to animal feed regulations. In comments filed with FDA recently, the groups said safeguards are already in place to protect the US livestock industry from the threat of bovine spongiform encephalopathy (BSE or mad cow disease).

BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) AND VARIANT CREUTZFELDT-JAKOB DISEASE (vCJD) IN HUMANS "The infectious agent of mad cow disease remains infective even after exposure for an hour to a temperature of 680 degrees Celsius - enough to melt lead - and can withstand antibiotics, boiling water, bleach, formaldehyde, and a variety of solvents, detergents and enzymes known to destroy most known bacteria and viruses." Rampton, Sheldon, and Staubcr, John, "Mad Cow U.S.A.: Could the Nightmare Happen Here?' PR Watch; See also Institute of Food Science and Technology (UK), "Bovine Spongiform Encephalopathy (BSE): part 1/6, part I of a 6-part position paper.

'Mad Pet' Disease: Mad Cow Disease is Killing Europe's Pets Autumn, 2001 Earth Island Journal Vol. 16, No. 3 by Ann N. Martin. In 1985, the world began to hear about a disease that was affecting cattle in the UK - bovine spongiform encephalopathy (BSE), or mad cow disease. Variants of the disease - known to affect cattle, sheep and humans - left the brains of its victims riddled with holes and resembling a sponge. Contaminated material from cattle infected with BSE has been rendered and used in commercial pet foods in Europe. Most scientists determined the practice would be safe; that BSE would not cross the species barrier. They were wrong.

Now fish too can suffer version of mad cow disease London, Feb 2 (ANI): Now fish like sheep, elk and humans could suffer a version of mad cow disease, or BSE, according to preliminary evidence. The results might help reveal how the disease jumps from species to species. The discovery of a prion in fish - which are evolutionarily distant from humans - suggest that the protein is "probably doing something fairly important and basic", says Jonathan Weissman, who studies protein folding at the University of California in San Francisco. (ANI)

The UK Creutzfeldt-Jakob Disease Surveillance Unit 

UK BSE Inquiry

BSE Review

UK Food Standards Agency on BSE

The Official Mad Cow Disease Home Page

Guardian(UK) on BSE

Wikipedia: Prions

Mad Cow Disease

2001/02/: Decontamination of Surgical Instruments and Other Medical Devices   

What Canadians Need to Know About Mad Cow Disease

NCBA: BSE Information 

USFDA: Bovine Spongiform Encephalopathy (BSE) Also Known as 'Mad Cow Disease'

2003/11/06: NEJM: Extraneural Pathologic Prion Protein in Sporadic Creutzfeldt-Jakob Disease

2004/01/29: Eureka: A new twist on the mad cow
Scientists at The Scripps Research Institute discover the normal prion protein may contribute directly to disease

 

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