Mad Cow Disease

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Bovine spongiform encephalopathy

Bovine spongiform encephalopathy is better known as Mad Cow Disease. The condition is caused by aberrant pathology in the central nervous system of cows, leading to the brain tissue becoming spongy in appearance. The specific pathology is a particular protein, a prion, no longer folding correctly and being able to cause other prion proteins from not folding properly, leading to a pathological cascade. Holes form in the brain, causing the spongy appearance.

Prions are a new kind of pathogen, and because of increases in a human variant of the disease during outbreaks of mad cow, there are concerns about sick cows and the food supply. Here's information on the progress of our understanding of this disease.

Timeline of BSE

  • 1920s: A human spongiform encephalopathy is discovered by two neurologists, Hans Creutzfeldt and Alfons Jakob. The rare disease occurs sporadically in the human population.
  • 1986: The first case of BSE is documented in British cows.
  • 1993: Peak of the BSE outbreak in the United Kingdom.
  • 1996: First case of variant Creutzfeldt-Jakob disease in humans in the UK
  • 1997: The Nobel Prize in Physiology or Medicine is awarded to Stanley Prusiner for his recent work in the discovery of prions as the infectious agent underlying spongiform encephalopathies.
  • 2003: BSE is documented in the United States.
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Mad cow in the UK

Mad Cow postage stamp from England, by Michaelfthompson, Wikimedia

Starting in 1986, mad cow disease ravaged the beef population of the UK. One commemoration of this impact was via the postal stamp.

Infectious parts of the cow

  • Offal - the discarded parts during the butchering process, such as the organs and entrails (varies by culture and mad cow has results in a change in some menu items in the UK)
  • Central nervous system - the brain and spinal cord
  • Meat that has come into contact with the central nervous system during the butchering process

Mad cow and the food supply

The fear of mad cow disease lies in its transmission to humans from eating infected meat. Prions are resistant to heat and little is known about them. An increase in the rate of variant Creutzfeldt-Jakob disease (CJD) in Britain has raised questions about the association between human and bovine disease. Proper butchering techniques that prevent the nervous system organs and those known to contain the prions from coming into contact with the edible parts should be enough to prevent the spread. As such, cows that exhibit any of the symptoms of the disease are supposed to be removed from the food chain and incinerated. It is also illegal to feed cows the parts of other animals, called ruminant feed and bone meal.



The last known cases of BSE in cows in Canada or the United States was as recently as February 2011 according to the Centers for Disease Control. Though Reuters reported on Twitter on April 24, 2012 "USDA confirms case of Mad Cow Disease found in dairy cow in Central California". Bloomberg reports that it did not enter the food supply. More info, including updates, is available in my blog post.

Mad Cow USA: Could the Nightmare Happen Here?

Amazon Price: $10.00 (as of 06/02/2012)Buy Now

Considered by many as a must-read to understand the fears underlying BSE

Resources

NOVA Online | The Brain Eater
Traces the scientific work that links a bovine brain disorder to human neurological disease.
CDC - Prion Diseases
Transmissible spongiform encephalopathies
WHO | Variant Creutzfeldt-Jakob disease
About human spongiform encephalopathy
FDA - About BSE
Article explaining BSE and what the FDA is doing to keep your food safe.
Spongy bovine brain from BSE prions

Spongy brain 

More information

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Mad cow in the news

Why BSE went so much better than pink slime
When a new case of bovine spongiform encephalopathy (BSE) was reported by the media in late April, the headlines weren't too bad. ?They may even be accused of being fair and accurate,? quipped Janie Gabbett, executive editor of Meatingplace, ...
California BSE case proves food safety system works
While the California dairy cow that tested positive for bovine spongiform encephalopathy, or mad cow disease, made national headlines, University of Georgia livestock and food safety experts say the real story is how well the nation's food safety.
Two California dairies released from BSE quarantine
Two California dairy facilities associated with the nation's fourth case of bovine spongiform encephalopathy (BSE) have been released from quarantine, says the US Department of Agriculture's (USDA) Animal and Plant Health Inspection Service (AHPIS).
Latest BSE finding in the U.S. should have little impact on markets
When the first case of Bovine Spongiform Encephalopathy (BSE) was reported in the United States in 2003, USDA figures indicated beef exports fell by more than 80 percent overnight in reaction to the confirmed case on a Washington dairy farm.

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Comments

  • Inkhand Jan 27, 2012 @ 7:30 am | delete
    Interesting lens, good thing I don't eat red meat.

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AliciaMae

Hello! My name is Alicia. I have a PhD in Experimental Pathology and am a freelance writer and editor specializing in scientific manuscripts and public... more »

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Giant Microbes Mad Cow (Bovine Spongiform Encephalopathy)

Amazon Price: $3.00 (as of 06/02/2012)Buy Now

A cuddly learning tool that allows you to get close to the spongiform encephalopathy pathogens without the disease!