Prion Deletion and Insertion Mutants

New claims link CJD to water supply
CJD geographical clusters?
Interview with Clare Tomkins' family
Is beef going to be what's for dinner now?
Fear emerges in Britain of disease time bomb
Mutation could lead to more lean meat, caesarians

New claims link CJD to water supply

August 26 1997  London Times Robin Young
Allegations about a rendering plant have raised fresh fears about the risk of infection 
AN INVESTIGATION into a cluster of CJD cases in Kent has led to fresh allegations that the domestic water supply could be a possible source of infection. A former building contractor at a plant rendering the carcasses of slaughtered cattle which may have been infected with BSE claims that liquid waste was poured down a well. The plant has strongly denied the allegation.

Alan Colchester, consultant neurologist at Guy's Hospital in London, who has treated three of the new-variant CJD cases from Kent, has already voiced his concern over a possible link with infected water. He said yesterday that he was anxious to know whether effluent from the rendering process, leaked into the water supply, could be a route of infection.

The former contractor, Gary Skillet, says workers at Canterbury Mills, a rendering factory near Godmersham, used a well to get rid of liquid waste from carcasses disposed of under the Government's culling programme. Speaking to Meridian Television's Meridian Focus, to be shown tonight, Mr Skillet said that effluent was regularly pumped into the 50ft well, providing a link to the aquifer which supplies domestic water to a large area of east Kent, where there have been three deaths from the new variant of CJD. All lived within 20 miles of the rendering plant.

Mr Skillet's claim comes days after the 22nd victim of new-variant CJD was identified. The case of Clare Tomkins, 24, from Tonbridge, in a different water area, 25 miles to the west of Godmersham, has raised fears about sources of infection because she has been a vegetarian since 1985, before the first case of BSE.

Mr Skillet, of Shadox, Kent, now a business studies student, said: "Basically, they put down the well whatever they could get down it. They used an electrically operated pump ... the well was used with everybody's knowledge to pump away materials they could not get rid of any other way."
The plant is understood to be the only one in Britain to spread its effluent across fields above an aquifer from which drinking water is taken. This fact, which became known in July 1996, so shocked Dr Colchester that he arranged a meeting with the Department of the Environment.
"I later discovered that, within hours of the meeting, a key part of the evidence had on the instruction of the Environment Agency been removed by blocking off a pipe," he said. "The Environment Agency undermined my confidence in how thoroughly they were carrying out the investigation.

"In my opinion it is very likely that the new-variant CJD is BSE in man. That is, it is transmitted from cattle to man. The route of infection has not been proven, but I think it is most likely that it is by the oral route, which is the greatest risk. If it was through liquid, then it would probably be suspended particles within water."

The Environment Agency strongly denied any impropriety:
"At no time has the agency concealed evidence or misled anyone over our actions at Canterbury Mills. Analytical results of samples taken from the well were made available to the public and remain so. We commissioned an independent risk assessment into Canterbury Mills and the well contents posed a negligible risk to public water supplies or human health."
David Richardson, director of Canterbury Mills, said: "We have never put anything down the well other than rainwater."

Water authorities issue denials

August 29, 1997
PA News
Jo Butler
Allegations have surfaced that five people in the Kent, U.K. area have developed nvCJD -- three within 20 miles of a Canterbury Mills cattle rendering plant that dumped liquid waste from cattle carcasses into a well near Godmersham, Kent, possibly polluting the water supply with what this story calls BSE infected material.

In response, industry leaders held an emergency meeting in London and declared the allegations "completely unfounded" and insisted supplies were safe to drink.

Richard Hampshire, Mid Kent Water's quality assurance manager was cited as telling members of the Water Companies Association there was no possible connection between the water supply and the victims, adding the water in the aquifer served by the well in question was only pumped to Ashford where there has been just one confirmed case of nv CJD.

Further, the story says there was no evidence that Canterbury Mills, the rendering plant which has always denied dumping any waste into the well, has ever handled BSE infected cattle.

CJD geographical clusters?

Kent:

1.  Anonymous (No. 4 in list of 19 Oct 96) 
2.  Anna Pearson, 29, Canterbury
3.  Barry Baker, 29, Ashford
4.  Graham Brown, 36, Ashford
5.  Elizabeth Bottle, 59, Ashford (possibly not nvCJD)
6.  Sue Carey, 36, Mersham near Ashford
7.  Clare Tomkins, 24, Tonbridge


Nothumberland/Durham:

1.  Peter Hall, 20, Durham
2.  Jean Wake, 38, Washington, Tyne and Wear
3.  Mandy Minto, 27, Sunderland


Scotland:

1.  Janice Stuart, 34, Glasgow
2.  Helen Rutherford, 15, Glasgow area
3.  Donna-Marie McGivern, 15, Coatbridge near Glasgow
Question mark on Kent # 1. The age is correct, uncertainty with Canterbury or Kent. Stephen Churchill, the first person confirmed as dying from nvCJD, often spent holidays at his aunt's farm in Sissinghurst, about 6 miles from Smarden, Kent - where Vet. Colin Whitaker found the first BSE case in April, 1985.

Today's limited data are not enough to predict the future of an nvCJD epidemic. Pressed to predict the size of the nvCJD epidemic Prof. Adrian Smith, President of the Royal Statistical Society of the UK, answered "zero to millions". It's simply too early to predict the scope of the epidemic.

However, it's possible - but not sure - that we're actually seeing the first signs of an increase just now. As Charles Arthur recently wrote in The Independent: "In 1995 there were three deaths, in 1996 ten, and 1997 looks set to double last year's figure".

Today's official numbers from CJDSU are 21 UK cases, with one additional confirmed French case and three new British cases (not yet counted by CJDSU) - that's a total of 25 cases today. Nine nvCJD deaths until the beginning of June this year, compared to 10 cases for the whole year of 1996. 12 nvCJD cases reported for 1997 in early August.

The next Dept. of Health official CJD statistics update will be on Sept., 1997.

Interview with Clare Tomkins' family

26 Aug 1997 Charles ArthurIndependent newspaper
I spoke last week to Clare Tomkins's father. He was of course unhappy about the news but dealt with the media with poise. My sympathies to him and his family.

Clare, he said, had been a strict vegetarian since 1985. She would not even eat biscuits if they said they had animal fats on the label, and avoided foods containing gelatin. "It had to be vegetable fats," he said. The family used to joke about how difficult she was to feed. Before she became vegetarian, the family used to eat supermarket meats; "anything that her mother cooked, normal average foods.Supermarket foods, butchers' foods."

The onset of the disease may have begun in March 96, based on her fiance's estimation, though the family first really noticed it when she lost weight around October 1996. She became withdrawn, unsociable, and depressed, and also anxious. These symptoms were not recognised as nv-CJD for a long time. She was treated on the basis that she had a psychological illness, and even given ECT. She began to lose coordination, and eventually the psychologists turned to doctors who turned towards a diagnosis of nv-CJD, which was made by John Collinge of St Mary's (better known for other work on BSE/CJD).

By my count she is the 25th victim in Britain. The government figure of 21 goes up to the end of June, but since then a number of other cases have come to light which have been confirmed by competent doctors in the areas. I don't think they say "I dunno - CJD?" It seems more like a diagnosis of absolute last resort. Clare is still, by the way, alive.

As to infected cattle. I spoke to Roy Anderson, who wrote the Nature paper of Nature 382 779-788. He estimates that between the late 1970s and 1985, based on his modelling of the BSE epidemic, 40,000 subclinical BSE-infected cattle would have entered the food supply. For the succeeding year it would be another 30,000. That compares to the figure of a total 446,000 (with limits 440,000-580,000) subclinical animals entering the food supply 1985 - 1989 when the SBO ban came into [nominal] force.

Cross-comparing with the Cousens paper on "Predicing the CJD epidemic in humans" (Nature 385 197-198 1997), the figures of incubation period of 11-12 years and 10 or so cases with onset in 1996 gives low figures for the final epidemic. Unfortunately Simon Cousens is away at present so not able to talk further on his interpretation of the latest figures.

Clare Tomkins's case does seem consistent with BSE exposure before 1985 (she would have been a child) and incubation period of 12 years or so. Remember that the HGH incubation period has a mean of 13 years (Cousens quoting Brown P., Lancet 340 24-27 (1992)) so this is not unusual in that context.

The thing about this case is that is gives strong evidence of how long the incubation period can be - or is. In many of the other cases one could say that people were eating beef products during the "maximum danger" period. She clearly wasn't. As to handling animal feed: why aren't feed mill workers dropping like flies? The hypothesis is too weak to sustain. This looks like BSE exposure orally.

The case of the rendering mill in that area is very interesting. I have noted the clustering that Kari asked about. The Sunderland and Glasgow ones are odd. John Wilesmith of CVL denies that there was any clustering of BSE, though, early in the epidemic's course. Just a few early cases and then boom (to paraphrase him).

Is beef going to be what's for dinner now?

AP August 26, 1997 DALLAS -- Consumers are looking closer at meat these days following another E. coli outbreak and cattle ranchers are hoping Americans' love of beef will last through this latest contamination scare. Mad cow disease, which hasn't been reported in the United States, previous warnings about E. coli and years of hearing nutritionists warn against too much red meat have many shying away from beef already.
"I was in the grocery store the other day and didn't buy any hamburger. I thought why not just wait awhile," said Cameron Tyler of Boulder, Colo.
A poll conducted for Newsweek magazine last week as the E. coli outbreak was getting wide attention found that 41 percent of those polled less likely to purchase hamburger at grocery stores, and 54 percent less likely to buy hamburgers at fast-food restaurants. Many cattle ranchers agree that the highly publicized outbreaks could wind up diminishing American's appetite for beef.
"The stigma is always a concern," said Texas rancher Chaunce Thompson, a past president of the Texas and Southwest Cattle Raisers Association. "But I feel like the American people are smart enough to realize these are very isolated incidents."
During an E. coli outbreak in 1993 that sickened more than 500 hamburger eaters and killed three children, consumers turned their backs on beef. Consumption bottomed out at 61.6 pounds per person, according to the National Cattlemen's Beef Association.

The beef industry, which has struggled to hold its market share over pork and poultry in recent years, has become highly sensitive to reports of contamination and is hopeful the same thing won't happen this time around. Reaction has been less this time around. Livestock futures on the Chicago Mercantile Exchange hovered around 69.85 cents a pound before the USDA's recall on Aug. 18, when they plummeted to 66.42 cents a pound. The prices have been creeping back upward since.

"I think that consumers are used to hearing about once a week about a food scare. We've heard, 'don't eat chicken, don't drink water, don't eat strawberries, don't eat apples'," said Lisa Williams, a spokesman for the Texas Beef Council. "Of course we're concerned about the recent E. coli outbreak, but we think consumers are starting to understand."
The Newsweek poll of 501 adults was taken Friday and has a margin of error of plus or minus five percentage points. It found that 51 percent of respondents in recent days had decided to avoid certain foods or were being more careful about handling and preparing food.

Fear emerges in Britain of disease time bomb

August 27, 1997
By OWEN BOWCOTT, The Guardian
LONDON -- Evidence of a long incubation period during which the new variant of Creutzfeldt-Jakob disease may develop undetected in the human body has reignited fears of a future epidemic. The case of Clare Tomkins, 24, a strict vegetarian for 12 years, raises the possibility that the small number of deaths recorded so far may merely reflect infection from before bovine spongiform encephalopathy (BSE) -- or "mad cow" disease -- was officially identified in cattle.

The diagnosis of Tomkins, from Tonbridge in Kent, in southeast England, who is receiving treatment at St. Mary's hospital in London, has not cast doubts on the links between BSE and the new variant CJD. But her case, the twenty-second in Britain so far, has prompted suggestions that the infection could be passed on by milk, cheese or gelatin. Tomkins, who was last year diagnosed as suffering from acute depression, gave up meat in 1985. The first cases of BSE were not confirmed in cows until the following year.

"Scientists are in no doubt that she caught CJD from mechanically recovered food eaten before 1985,"' said her father, Roger Tomkins. "If my daughter was infected before 1985, then we have a CJD time bomb within the country."
John Pattison, chairman of the British Government's Spongiform Encephalopathy Advisory Committee (SEAC), admitted that the case showed the longest incubation period. "The fact that this woman was a vegetarian for such a long time is an unusual feature and we will think about what the implications for that are,"' he said. Evidence discounting milk or cheese as an infectious agent should be re-assessed, he added. Professor Richard Lacey, who first highlighted the BSE threat, warned that scientists should not dismiss the possibility that drinking milk, with low concentrations of the agent over a long period, might be the same as eating highly infected meat.
"The experiments (using milk) are not satisfactory,"' he suggested. "It just means you can't pump enough infected milk into a mouse. But milk might be infectious if consumed over a long period."
Peter Smith, an epidemiologist at the London School of Hygiene and Tropical Medicine and another member of SEAC, said the relatively low rate of new diagnosed cases made estimates of
"millions of future victims look rather implausible. I think the final toll will be at least some hundred or more. I would be much more concerned if there had been a rapid increase over the last year. More cases are going to occur for some years, even decades, to come."
Four of the victims' families have been granted legal aid to sue the Ministry of Agriculture, Fisheries and Food for negligence in not preventing the infection passing to humans. The families' support network is pressing for the new variant CJD to be renamed Human BSE, said Dot Churchill, whose 19-year-old son Stephen was among the earliest victims in 1995.
"All the families want some form of independent inquiry into what's happening. But both the last government and this one have refused," she said.
The long incubation period in Tomkins's case suggests that however large the outbreak may eventually be, it has probably not peaked.

Gene mutation could lead to more lean meat

By TIM RADFORD, The Guardian August 27, 1997 
Scientists have identified the gene which makes some cattle grow more muscle, enabling them to provide more lean red meat. The discovery, reported Tuesday in the journal "Genome Research," could open the way for new methods of cattle breeding.

The mutation is a natural one, and the discovery is hailed as the first identification of the DNA behind "agriculturally desirable traits." But the meatiest cattle pay a heavy price for all that beef: calves may be too big to be born naturally.

The research began with a mouse when scientists at Johns Hopkins University were trying to understand something about muscle-wasting diseases like muscular dystrophy. They took a gene called growth differentiation factor 8 (GDF8) out of the mouse -- and turned the creature into a massively-muscled version of itself.

GDF8 exists in many animals. Ravi Kambadur at the Ruakura Research Institute in New Zealand and Timothy Smith of the U.S. Agricultural Research Service in Nebraska identified mutations in the GDF8, or myostatin gene that make some cattle -- such as the double-muscled Belgian Blue -- have less bone and fat and 20 percent more muscle.

The Belgian Blue is a breeding accident. "The mutation has naturally occurred. There was no need to manipulate the genome,"' said Smith. This mutation is a mixed blessing. Charolais breeders would like to be able to test for the mutation and eliminate it.

"The meat is much more lean,"' he said. "But the limb bones are a little bit less stable and in the Belgian Blue breed and the Charolais they have of problems with birth, because the calves are so big."
One solution might be to manipulate the gene so that animals grow large and beefy -- but only after they had been born.

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