Jerusalem Post July 2, 2001

Hadassah team identifies 'mad cow' protein

By Judy Siegel

JERUSALEM (July 2) - A Hadassah-University Hospital research team has discovered that the prion (protein particle) responsible for the fatal "mad cow disease" in animals and Creutzfeldt-Jakob disease in humans can be detected in urine.

The tiny pathogen that causes the incurable neurodegenerative disease bovine spongiform encephalopathy (BSE) and its human variant can be identified even before symptoms appear. This means that blood donations by human carriers can be prevented, and millions of unaffected cows will be saved from wholesale slaughter.

The groundbreaking article on the discovery has just been published in the Journal of Biological Chemistry by Dr. Ruth Gabizon of the neurology department at the Ein Kerem hospital and her team of Gideon Shaked, Yuval Shaked, Zehavit Kariv, Michele Halimi, and Inbal Avraham.

The only known component of the prion, known as PrPSc, is found mostly in the brains of animals and humans affected with prion diseases, and has not been detected in blood. But the researchers demonstrate that a substance called protease-resistant PrP isoform can be detected in the urine of infected hamsters, cattle, and humans.

Gabizon said yesterday the most important thing is that "the PrP isoform was also found in the urine of hamsters inoculated with prions long before the appearance of clinical signs." Over 100 people have died in Europe, mostly in England, in recent years after eating beef infected with BSE, and many more are expected to fall victim to it, as the disease can be carried for decades before symptoms appear.

The disease was first discovered in England in 1985. Cows contracted the disease after eating feed made from sheep infected with scrapie. Since then, millions of cows have been destroyed in England and some other European countries, nearly wiping out their cattle-raising industry. Although it has not yet been proven that blood from people who carry Creutzfeldt-Jakob disease can pass the human variant on to others in infusions, the possibility has raised much concern.

Gabizon said that almost no one has looked at the urine for prions because it was thought prions did not pass through the kidneys.

Since last September, Gabizon and her doctoral student Shaked had been looking for other substances in hamster urine. They identified the tiny protein particles, and found that they do not break down in the kidneys but are eliminated in the urine.

Gabizon and her team are in the final stages of establishing a Jerusalem start-up under the auspices of Hadassah's research and development arm Hadassit to produce a commercial kit for testing animal and human urine.

So far, BSE has been easily diagnosed using urine taken from over 50 British cows diagnosed with BSE and sent to Israel, along with samples from healthy cows, by the Veterinary Laboratory Agency. "In the blind test, we quickly picked out the affected animals," Gabizon said.

A decade ago, Gabizon worked in the lab of Prof. Stanley Prusiner, the American discoverer of the prion who recently received the Nobel Prize for Medicine for the achievement. Since she returned to Hadassah, she has dedicated her time to work on prions.

Currently, people who have spent even a short time in England are barred from donating blood. However, when the urine test becomes available, this sweeping restriction will most likely be dropped.

Gabizon said the Hadassah discovery also raised the "alarming possibility" that the human and animal forms of the disease can be transmitted by contact with infected urine, even from those who as yet show no symptoms.

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© 1995-2001, The Jerusalem Pos

-- Lars (larsguy@yahoo.com), July 02, 2001

Answers

How do you make a cow piss in a bottle?

-- (nemesis@awol.com), July 02, 2001.

This is good news, Lars. It seemed stupid to me to simply slaughter cattle because they were suspect. I'm really hoping that more tests like this can be developed for humans. My oldest brother finally went in for his first colonscope. He hadn't gone in for the routine tests like my other brother and I. He waited until a fecal test showed blood in his stools. Sure enough, he has colon cancer. They removed part of his colon and think they got it all, but I REALLY want a test like this one for MY children and the other children in my family. We're all suspect.

-- Anita (Anita_S3@hotmail.com), July 02, 2001.

The tiny pathogen that causes the incurable neurodegenerative disease bovine spongiform encephalopathy (BSE) and its human variant can be identified even before symptoms appear. This means that blood donations by human carriers can be prevented, and millions of unaffected cows will be saved from wholesale slaughter.

Both of these outcomes are very good. In the meantime, I only eat beef that is welldone.

-- Lars (larsguy@yahoo.com), July 02, 2001.

I'm not as cautious as you, Lars. I don't enjoy ANY meat that's well- done. [There's that Hannibal Lecter part of me coming out again.] I don't even cook pork until it's well-done. In my younger days, I would say, "Have you ever heard of ANYONE who died from tularemia?" Folks would respond with, "People don't die of it anymore because they all KNOW to cook it thoroughly, Anita."

-- Anita (Anita_S3@hotmail.com), July 02, 2001.

I love you Lars and Anita. Forgive me for being the bearer of bad news.

Prions are not destroyed by cooking. The temps required to destroy prions are higher than is normally reached by an autoclave. This means that prions are not only spread by eating cooked meat, they are also theoretically spread by dental instruments. (Several cases of hepatitus have been trace to colonscope equipment. Prions are even tougher than hep germs.)

The good news is, after a point you won't even know you have BSE. It won't hurt you, just your loved ones who have to watch.

-- helen (be@ring.bad.news), July 02, 2001.

Helen, what a cheery piece of news. I think well-done is still a protection against salmonella.

Anita, are you into raw chopped beef with raw onion? (Big in Milwaukee, a German thing)

-- Lars (larsguy@yahoo.com), July 02, 2001.

Using dental instruments in a coloscopy is not recommended.

-- (nemesis@awol.com), July 02, 2001.

Anita, are you into raw chopped beef with raw onion? (Big in Milwaukee, a German thing)

Nope. I enjoy beef cooked medium/rare and pork cooked medium. I HAVE heard of folks who enjoy raw chopped beef on celery. I've also heard of folks who enjoy peanut butter on celery. I don't much like celery ALONE, let alone mixed with this other stuff.

I DO want to say, "I love you, Lars." Laundry is a tedious chore to me, but you've spared me the tedium. I also love YOU, Helen. Your posts never cease to amuse/teach me.

-- Anita (Anita_S3@hotmail.com), July 02, 2001.

"they are also theoretically spread by dental instruments. (Several cases of hepatitus have been trace to colonscope equipment."

Colonscope does not sound like a dental instrument. It sounds like something completely different. Something I do not ever want in my mouth.

Deano

-- Deano (deano@luvthebeach.com), July 02, 2001.

I love you, Nemesis and Deano. You are both absolutely right. Keep all dental instruments out of your rectums. Keep proctology equipment out of your mouths. You'll still be exposed to prions, though. Did you ever see "Night of the Living Dead" with Clu Gulager?

-- helen (no@yucky.stuff.allowed), July 02, 2001.

Clu Gulager?! Now there's a name from the past, and a not very important one at that.

Wasn't he appropriated by prions a priori?

-- Lars (larsguy@yahoo.com), July 02, 2001.

The development of a test is great news, but there is this 'kicker' at the end of the article:

"Gabizon said the Hadassah discovery also raised the "alarming possibility" that the human and animal forms of the disease can be transmitted by contact with infected urine..."

If this test can be packaged economically, it will sell in the tens of millions. It should help slow the spread of the disease (woo-hoo!), but won't do a thing for the currently infected (bummer).

-- Little Nipper (canis@minor.net), July 02, 2001.

I'm confused by your reply, LN. I think the GOAL was to not kill the suspects. Once an organism has succumbed to a disease [be it animal or human], it will be destroyed [if not by the disease itself, by human intervention.]

Are you suggesting that terrorists will purchase the urine of infected animals and splash it upon the unsuspecting? I dunno. I just know that your reply confused me.

-- Anita (Anita_S3@hotmail.com), July 02, 2001.

Urine is an important ingredient in fertilizer. So is animal poop, which is doused in urine. We're doomed. This time instead of storing rice, why don't we have one hell of a fine party at my house?

-- old mchelen had a farm ... (eiei@o.oooooo), July 02, 2001.

Sorry if I didn't make my point clear.

A simple, widespread test should go a long ways toward identifying animals that must be destroyed to protect those who are not infected. That should help a lot toward controlling the disease, by pinpointing the carriers.

But the news that the urine of infected animals could spread the disease is ugly, since the more pathways there are for spreading the infection, the more likely it becomes that there are more human victims than currently believed.

There is no cure. It appears to be invariably fatal. Being able to test for the disease won't cure any person who already has it. Under current conditions, they are pretty much doomed.

Also, it has a very long latency period, so there could be a lot of victims out there who don't know they have the disease. That, together with the fact that contact with human urine is fairly common event, so this suggests that eradicating the disease among cows may not be enough to keep the disease in check. This is the first credible pathway from human to human (other than cannibalism).

Bad news, that.

-- Little Nipper (canis@minor.net), July 02, 2001.

That, together with the fact that contact with human urine is fairly common event,

Forgive me, LN. You must know by now that I have a VERY low IQ. I STILL love you, however.

I'm trying to think of the last time I came in contact with human urine [other than the swipe I take with the toilet paper each time I urinate.] Not only can I not remember the LAST time, but I can't remember the FIRST time [outside of changing the diapers of my kids.] I know my memory is poor, but could you give me some examples of when/where I may have come in contact with human urine?

-- Anita (Anita_S3@hotmail.com), July 02, 2001.

I take a "golden shower" weekly.

-- (nemesis@awol.com), July 02, 2001.

"I'm trying to think of the last time I came in contact with human urine"

In hospitals, nursing homes, in homes with infants or with the disabled, or in some other lines of work (think janitorial services or working in a tavern), contact with urine is pretty "common". No one is supposed to treat that casually, but... here's a damn good reason to treat it more seriously.

-- Little Nipper (canis@minor.net), July 02, 2001.

Ah so, urine therapy.

-- (Paracelsus@Pb.Au), July 02, 2001.