Origins of mad cow disease

Posted on January 17, 2020 by Douglas Powell

Aristos Georgiou of News Week reports an international team of scientists has said that they may have identified the origin of mad cow disease. Known as bovine spongiform encephalopathy, the neurodegenerative disease destroys the brain and spinal cord in cattle, causing death.

Since BSE first appeared in the 1980s in the United Kingdom, scientists have tried to identify how the disease emerged, however, no one hypothesis has been confirmed.

For a study published in the journal Proceedings of the National Academy of Sciences, the team of scientists investigated the origins of BSE by injecting a particular variant of scrapie disease into mice which have been genetically modified with bovine DNA.

The researchers say that, unexpectedly, the injection of the scrapie strain into the genetically modified mice resulted in the propagation of classical mad cow disease prions. These prions are present in natural form in the scrapie variant.

This observation indicates that the illness could be transmitted between different species and that the modified mice could develop mad cow disease, according to the study.

Olivier Andreoletti, an author of the paper from the French National Institute for Agronomic Research (INRA,) told AFP that the modified mice are “a very good model, which works well in terms of knowing what would happen if one exposed cows to those prions.”

He noted that the results provide, for the first time, and “experimentally underpinned explanation” for the appearance of mad cow disease in the U.K. in the 1980s.

After emerging, the disease spread in cattle across Europe, North America and other regions of the globe. This process was exacerbated by the fact that cows were being given feed which contained tissue from other cows infected with the disease.

CJD sucks: UK special forces legend who blew up Saddam’s communications network dies of CJD

Posted on August 29, 2017 by Douglas Powell

A superfit, special forces legend from Gloucestershire who carried out a real-life “mission impossible” behind enemy lines has died after developing the human form of Mad Cow Disease.

Father-of-three Mark Phillips MBE was just 56 when he died earlier on August 12 and was well-know figure in military circles for his brave exploits in Afghanistan and Iraq.

Known as Lt. Colonel Mark ‘Foggy’ Phillips of the Special Boat Squadron, he is said to have being diagnosed with Sporadic-Creutzfeldt-Jakob Disease in June. It is so rare that only one in one million people develop it.

It is believed that Mr Phillips attended Balcarras School in Cheltenham before embarking on an outstanding military career which saw him join the SBS in 1987 and become one of its fittest and most respected officers.

Commandant General of the Royal Marines, Major General Rob Magowan, led the tributes to the officer who is said to have carried out hundreds of forays behind enemy lines, including an audacious mission to blow up Saddam Hussein’s telecommunications cables buried under a sports arena near the centre of Baghdad.

Sources say diversion was created so Lt Col Phillips and his SBS team could fly in on Chinooks at low altitude to avoid the radar and then then plant hundreds of pounds of explosives.

An SBS source told the Daily Mail that many of those in the know did not expect them to make it back alive from the 1991 raid because it was the “It was the proverbial ‘mission impossible’.”

But it was so successful in bringing down Iraqi communications during the first Gulf War that a piece of cable recovered from the scene was later put on display at the Imperial War Museum.’

The military man returned to Iraq in 2003 with the SBS and in 2008 he was said to have joined a special UK-US Special Forces unit known as Task Force 42 which tracked Taliban commanders.

Major General Magowan said: “Foggy was an inspiration, both to me and across our Corps. Bright, physically strong, courageous, hugely visionary and immediately engaging, he had all the attributes of a Royal Marine.

“People were swept up by his energy and leadership. I first met him on an adjacent rowing machine and I must admit to feeling intimidated. As an organisation, we are considerably less rich with his passing.”

Although he kept out the public eye, he was also known as an athlete and the 1990s he won the 125-mile Devizes to Westminster canoe race, which has previously won by Paddy Ashdown and Ranolph Fiennes, four times in succession.

Organisers paid tribute to him on the event’s Facebook page and said: “His athletic feats are legend and amongst these achievements, he was a 4 times winner of the Devizes to Westminster canoe race.

“Lt Col Mark Phillips MBE, Royal Marines was a professional of the highest order – we will be hard put to meet his like again.”

According to his LinkedIn profile he left the military in 2013 and had his own security business.

A death notice in GloucestershireLive read: PHILLIPS M.B.E. Mark Sadly passed away on 12th August 2017 after a short illness. “Husband to Jacqui and father to Emily, George and Bethany. Son of Brian and Pat and brother to Stephen and Adrian. Funeral service will take place at Milton Abbey, Dorset on 25 August.”

Beneficial role clarified for brain protein associated with BSE

Posted on August 9, 2016 by Douglas Powell

Studying mice and zebrafish, researchers from Washington University School of Medicine in St. Louis and the University of Zurich have shown that the proteins — when properly folded — play a vital role in nerve cell function by maintaining the insulation around axons, the nervous system’s electrical “wiring.”

The study appears August 8 in the journal Nature.

Improperly formed prion proteins that cause disease are infectious because they hijack their neighbors, resulting in misfolded proteins and setting off a domino effect that spreads through the brain destroying tissue. Although the role of prion proteins in these fatal brain diseases is well-known, scientists have long puzzled over the normal function of the protein, called PrPC.

“Previous studies have suggested a role for prion proteins in maintaining neurons, but until now, no one knew how the properly folded versions of the proteins function,” said co-author Kelly R. Monk, PhD, an associate professor of developmental biology at Washington University. “It’s surprising to see that the protein has a role in maintaining the structure of nerve cells, considering that a misfolded version of PrPC is known to cause fatal brain diseases.”

Past work by the researchers at the University of Zurich demonstrated that mice lacking PrPC had disruptions in the insulation surrounding axons, but the reasons for the disruptions were unclear. The new study demonstrates that PrPC binds to Schwann cells, which are cells that provide support for the brain’s neurons. Schwann cells produce the nerve-insulating protein called myelin and then wrap this insulation around the long, thin axons. Properly insulated axons enable the rapid propagation of nerve signals. Specifically, PrPC binds to a docking site on Schwann cells called Gpr126.

In past work, Monk and her Washington University colleagues demonstrated that the docking site on cells played an important role in nerve formation during embryonic development in zebrafish and in mice. But the new study identifies roles for both Gpr126 and PrPC in maintaining the integrity of neurons through adulthood.

When either of these components is missing, Monk said mice experience a gradual loss of interactions between Schwann cells and axons, with a resulting loss of of myelin. Without this important insulation, walking progressively becomes more difficult for mice, and they eventually reach a state of paralysis.

“We have identified a definitive function for the normal prion protein and clarified how it works on a molecular level,” said senior author Adriano Aguzzi, MD, PhD, of the University of Zurich. “Our study answers a question that has been intensely researched since the prion gene’s discovery in 1985.”

The researchers said the findings may have implications for understanding and eventually treating nerve disorders that result from the loss of the insulating myelin sheaths, such as Charcot-Marie-Tooth disease and other devastating peripheral neuropathies.

Could Brexit make British food worse than mushy peas and mad cow disease

Posted on June 30, 2016 by Douglas Powell

Fortunately I live in the sub-tropical climate of Brisbane, with an outstanding variety of food.

But I grew up in Canada, and cherished the orange in my Xmas stocking.

Now it’s routine.

Has no one seen Pirates of the Caribbean, terrible movies that make gazillions and it’s about conquest and trade, because British food royally sucks.

I try to mention that every time someone preaches about eating local: coffee and tea are not local to Canada or Britain. Neither are bananas.

Bee Wilson writes in The New Yorker that for most of her 42 years, she has been eating bananas in Britain.

The classic anti-European Union joke was that faceless Eurocrats banned “wonky” bananas and imposed a single, standardized fruit on the British people. Bureaucracy gone crazy! This Euromyth—one of many—was based on a 1994 E.U. ruling that bananas should be “free from abnormal curvature.” In fact, this rule applied only to bananas of the very highest grade. The normal British Class I and Class II bananas sold in most shops have always been allowed “defects of shape.”

Now that the Leave campaign has won the referendum on Europe, it is clear that far more was at stake for British food in the E.U. than our right to misshapen fruit.

On June 24th, Tim Lang, a professor at City University and the leading U.K. food-policy expert, tweeted, despairingly, “EU shock. Very sad.” And then, “Food Plan B now needed. Will the people who voted Brexit be prepared to dig for Britain, work in picking fields and factories for low pay?”

One of the main reasons for establishing the E.U. in the first place—aside from peace—was to insure a plentiful food supply for entire populations.

As Lang sees it, Europe not only nourished the British but changed British palates. With Britain’s membership in the union, sunny new produce flooded in from southern Europe: apricots, peaches, tomatoes, garlic. During the decades of our membership, the food on British dinner tables changed beyond all recognition. We developed a penchant for wine and soft French cheeses. In 1973, the U.K. was a country where olive oil could be bought—if at all—in tiny bottles from the chemist shop, as a cure for earwax. Now you could get lost in the olive-oil section of a British supermarket, from the kalamata varieties of Greece to the Arbequina of Spain.

In April, Lang co-authored—with Victoria Schoen—a “briefing paper” on the far-reaching impact of Brexit on British food. Lang and Schoen point out that, as of 2015, twenty-seven per cent of all food eaten in the U.K. (by value) was imported from the E.U. (compared with just four per cent from North America and four per cent from Africa). When it comes to fruits and vegetables, Britain is dependent on the E.U. for forty per cent of fresh produce. Lang sees this as a question of health as much as economics.

As Lang and Schoen write, “A vast array of agreements, policies and standards now underpin UK food.” Brexit could entail the renegotiation of thousands of exceedingly complex E.U. regulations, many of which concern the food system. E.U. law extends from environmental law and farm subsidies to food safety and nutrition. Brexiteers would say that it is precisely this complexity that Britain could do without.

Now, about the safety of that food.

News amplification: Mad cow disease far greater impact on beef purchases than E. coli

Posted on May 18, 2016 by Douglas Powell

In December 2003, Bovine Spongiform Encephalopathy (BSE) was discovered in the United States. This food safety event received extensive media coverage and prompted changes in regulatory controls.

Using a panel selection model, we show that prior to December 2003, ground beef recalls had no impact on household purchases of ground beef, even for households that were located in the recall-defined geographic areas. However, we find robust evidence that the 2003 BSE event caused a change in the way people view and respond to recalls of ground beef, a change that persisted for at least two years following the BSE event.

The average impact of a ground beef recall in the post-BSE period is a 0.26 lb per person reduction in retail purchases of ground beef. A decline in purchases of this magnitude would result in over $97 million in losses to the beef industry in a two-week period following a nationwide recall.

This dwarfs the economic impacts of directly removing recalled beef from supply chains and provides FSIS increased regulatory power due to higher overall industry costs associated with food safety violations.

Changes in U.S. consumer response to food safety recalls in shadow of a BSE scare

Mykel Taylora, H. Allen Klaiberb, Fred Kuchlerc

Food Policy, Volume 62, July 2016, Pages 56-64, doi:10.1016/j.foodpol.2016.04.005

http://www.sciencedirect.com/science/article/pii/S0306919216300239

Confirmed variant Creutzfeldt-Jakob disease (vCJD) case in Texas

Posted on June 4, 2014 by Douglas Powell

Laboratory tests have confirmed a diagnosis of variant CJD (a fatal brain disorder) in a patient who recently died in Texas. The confirmation was made when laboratory results from an autopsy of the patient’s brain tested positive for variant CJD.

First described in 1996 in the United Kingdom, variant CJD is a rare, degenerative, fatal brain disorder in humans. It is believed to be caused by consumption of products from cows with the disease bovine spongiform encephalopathy (BSE, or mad cow disease).

Worldwide, more than 220 variant CJD patients have been reported, with a majority of them in the United Kingdom (177 cases) and France (27 cases). This case is the fourth to be reported in the United States. In each of the three previous cases, infection likely occurred outside the United States, including the United Kingdom (2 cases) and Saudi Arabia (1 case). The history of this fourth patient, including extensive travel to Europe and the Middle East, supports the likelihood that infection occurred outside the United States.

The U.S. Centers for Disease Control assisted the Texas Department of State Health Services (DSHS)’s investigation of this case and will continue to help confirm further details of the patient’s history, including the potential source of infection.

A classic form of CJD, which is not caused by the BSE agent, occurs worldwide, including in the United States. Annually, for every 1 million people in the United States, 1 to 2 develops classic CJD.

Evans of CFIA gets honorary degree

Posted on April 12, 2014 by Douglas Powell

Dr. Brian Evans, former chief vet at the Canadian Food Inspection Agency and the point-man on the first mad cow disease outbreak in 2003, is the first recipient of an honorary degree in veterinary medicine from the University of Calgary.

Evans is the retired executive vice-president of the Canadian Food Inspection Agency and was the country’s chief veterinary officer for 15 years.

He graduated from the University of Guelph with a bachelor of science in agriculture in 1974 and a doctor of veterinary medicine in 1978.

While at CFIA, he was also Canada’s delegate to the World Organization for Animal Health (OIE) for 13 years. He was elected secretary general for the OIE regional commission for the Americas and to four consecutive three-year terms as the representative of the member countries of the Americas Region on the OIE’s executive council.

Most recently, he was appointed the international body’s deputy director general responsible for animal health, veterinary public health and international standards.

He is married to Laurianne and has four children.

And is a genuinely thoughtful dude.

Alberta’s cattle industry sort of recovers a decade after mad cow outbreak

Posted on May 19, 2013 by Douglas Powell

On March 20, 1996, British Health Secretary Stephen Dorrell rose in the House to inform colleagues that scientists had discovered a new variant of Creutzfeldt-Jacob disease (CJD) in 10 victims, and that they could not rule out a link with consumption of beef from cattle with bovine spongiform encephalapthy (BSE), also known as mad cow disease.

Overnight, the British beef market collapsed and politicians quickly learned how to enunciate BSE and CJD. Within days, the European Union banned exports of British beef; consumption of beef fell throughout Europe, especially in France and Germany, and in Japan, where suspicion of foreign food runs high. The triumvirate of uncertain science, risk and politics was played out in media headlines.

To refer to the events of 1996 as the BSE crisis is a misnomer, just as scientists are quick to point out that mad cow disease should more appropriately be called sad cow disease or unco-ordinated cow disease. Rather, the announcement of March 20, 1996 was the culmination of 15 years of mismanagement, political bravado and a gross underestimation of the public’s capacity to deal with risk. More important than any of the several lessons to be drawn from the BSE fiasco is this: the risk of no-risk messages. For 10 years the British government and leading scientific advisors insisted there was no risk — or that the risk was so infintesimly small that it could be said there was no risk — of BSE leading to a similar malady in humans, CJD, even in the face of contradictory evidence. The no-risk message contributed to the devastating economic and social effects on Britons, a nation of beefeaters, the slaughter of over 1 million British cattle, and a decrease in global consumption of beef, especially in Japan, at a cost of billions of dollars.

The Canadian Minister of Agriculture was quite adamant there was no risk of BSE developing in Canada.

In July 1996, Dr Norman Willis, Director General, Animal and Plant Health, Agriculture and Agrifood Canada, told the Canadian Veterinary Medical Association’s annual convention that “Actions were taken out of sheer paranoia, with people significantly hyped by the media. We took actions that went way beyond ones that were scientifically justified. … We wouldn’t have political interference. We wouldn’t have non-science factors influence the actions we took. BSE blew that all away. … Canada and other trading countries couldn’t hold with science-based decisions. There was just too much at stake by way of trade.’’

Canada’s initial dragging to the grown-up’s table of BSE risk management, and apparent lax enforcement of feed regulations for years afterwards led to the inevitable: On May 20, 2003, Canada announced its first home-grown case of BSE.

The eight-year old cow from Alberta had been condemned at slaughter, was sent for rendering and did not enter the food chain.

The first few chapters of the story about the discovery of BSE in Canada were positive.

BSE was bound to show up eventually and the surveillance system set up in 1992 sorta worked. The inspector who pulled a sickly looking eight-year-old cow from the slaughter line prevented it from entering the food chain.

The line in Canada was, this is not the UK, and I was on TV at 5 a.m. the next morning, saying the U.K., had some 186,000 cattle test positive and millions preemptively slaughtered. The significant question was, will Canadian numbers of BSE-positives remain in the dozens or the tens-of-thousands (or something like that).

And yes, producers, processors and government should have been fully aware of the risk rather than act stunned when it happened.

Ten years on, with the perspective that time often offers, my statements seem accurate but naïve.

Canada has since reported 18 cases of BSE, and, just like other aspects of food safety, those in charge talk a good line, but do they know what really happens on farms (or anywhere) day-in, day-out.

And are they interested? Because being interested costs money.

Ian Gray of the Edmonton Journal wrote a 10-year-retrospective piece on the first homegrown BSE case today, beginning with:

In January 2003, Marwyn Peaster’s cow fell down.

The six-year-old Black Angus was one of a small herd Peaster had bought the year before for his grain farm and feedlot near Wanham, in Alberta’s Peace Country.

Believing the cow had pneumonia, Peaster made the fateful decision to send it to a local abattoir instead of calling for a veterinarian or disposing of it on his own property. The vet at the slaughterhouse went by the book and condemned the “downer” cow, so there’d be no chance it could be used for human consumption.

The carcass then went to a rendering plant, but the head was sent to a provincial laboratory in Edmonton for testing. As there was no perceived urgency, there it sat, until May 16, 2003.

Three and a half months after it was shipped, the head was finally tested at the provincial lab and, to the disbelief and horror of everyone involved, registered positive for BSE. The results were confirmed by federal and international laboratories and were announced to the public on May 20.

As of May 1, 2013, vCJD had killed 237 people worldwide.

The attack on Peaster reached its peak that September with the now infamous remark by then-premier Ralph Klein that “any self-respecting rancher would have shot, shovelled and shut up, but he (Peaster) didn’t do that.”

Peaster has since moved back to the U.S. and is living in the farming community of Ontario, Oregon, where he has a small trucking company. True to form, he has no desire to comment on the 10th anniversary of the discovery of BSE in Canada, in which he was a key, if unwilling, player.

Mad cow no longer dominates the food safety headlines, and that’s good. The potential is always there, and requires good risk management, but a lot more people get sick from lots of other things associated with beef (although vCJD is a terrible way to die).

As the elementary school year wound down in June, 2003, in Ontario, Canada,  the school three of my four daughters attended had a barbeque for students, staff  and parents.  The earlier discovery of Canada’s first domestic case of bovine spongiform  encephalopathy which received  extensive media coverage, was of concern to some parents and school  officials, so a note was sent home to parents, assuring them that the  hamburgers and hot dogs to be consumed came from a supplier of so-called  natural, beef and was therefore safe from BSE.

At this particular BBQ, several of the well-meaning volunteer cooks were  observed to handle the raw, natural hamburger patties with tongs that were  then used to place re-heated wieners into hot dog buns, possibly  cross-contaminating the wieners with any number of pathogenic microorganisms such as E. coli O157:H7, salmonella or listeria, and subsequently served to  parents and children.

About the same time, a bunch of industry folks hosted a BBQ on Parliament Hill in Ottawa, to demonstrate the safety of Canadian beef to politicos.

I watched the servers cook burgers, not use thermometers, and cross-contaminate everything in sight.

I asked, where is the hamburger form?

Don’t worry, it’s not from Alberta, no mad cow here.

Are these pre-cooked?

Nah, they’ve been sitting in the (non-refrigerated) truck for a few hours.

I always wondered if anyone got sick after that feast.

Shoot, shovel and shut up – the wrong approach for animal and zoonotic diseases

Posted on November 1, 2009 by Douglas Powell

Daughter Sorenne woke up around 6:15 a.m. after a big Halloween night (thanks for the costume, Katie). Then the clocks on the computer changed and I realized it was 5:15 a.m.

Damn you daylight savings.

So while Sorenne plays on the floor and fills her diaper, I’m looking at a poignant release from the France-based World Organization for Animal Health, inexplicably referred to as OIE (it’s a French thing) reiterating the importance of animal health rules to control human disease.

When the first case of bovine spongiform encephalopathy or mad cow disease was discovered in Canada in May, 2003, Alberta premier Ralph Klein famously declared that any

"self-respecting rancher would have shot, shovelled and shut up."

In 1184, city leaders in Toulouse, France, introduced some of the first documented measures to oversee the sale of meat: profit for butchers was limited to eight per cent; the partnership between two butchers was forbidden; and, selling the meat of sick animals was forbidden unless the buyer was warned.

By 1394, the Toulouse charter on butchering contained 60 articles, 19 of which were devoted to health and safety.

As outlined by Madeleine Ferrières, a professor of social history at the University of Avignon, in her 2002 book, Sacred Cow, Mad Cow: A History of Food Fears, the goal of regulations at butcher shops — the forerunners of today’s slaughterhouse — was to safeguard consumers and increase tax revenues. Animals from the surrounding countryside were consolidated at a single spot — the evolving slaughterhouse, originally inside city walls — so taxes could be more easily gathered, and so animals could be physically examined for signs of disease.

It’s no different today: slaughterhouses are common collection points to examine animals for signs of disease and to collect various levies. And like medieval times, one of the most basic rules is animals that cannot walk are forbidden from entering (the slaughterhouse or city).

Bernard Vallat, Director General of the World Organisation for Animal Health (OIE), reminded the world this morning that veterinary legislation is the foundation of any efficient animal health policy.

Veterinary legislation is a critical infrastructure element for all countries. In many OIE Member countries, the veterinary legislation has not been updated for many years and is obsolete or inadequate in structure and content for the challenges facing veterinary services in today’s world.

Dr Vallat says that it is important that the veterinary services have the authority to enter livestock premises and other establishments and take the actions needed for early detection, reporting and rapid and effective management of any animal diseases as soon as they are detected. Such actions include the capacity to seize animals and products, to impose standstills, quarantine, testing and other procedures; to control animals and products at frontiers; and to require the destruction and safe disposal of animals and all articles considered to present a risk of disease transmission and to public health. These activities represent the core activities of veterinary services in the field of animal health control and veterinary public health and the legislation must provide the necessary authority as a minimum.

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