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http://www.upi.com/view.cfm?StoryID=20040727-020751-7281r

 

NIH to shut down mad cow lab

 

By Steve Mitchell

United Press International

Published 7/28/2004 9:59 AM

 

WASHINGTON, July 28 (UPI) -- The U.S. government

laboratory credited with conducting groundbreaking

work on human brain disorders similar to mad cow

disease soon will be shut down, United Press

International has learned.

 

The lab -- known officially as the Laboratory for

Central Nervous System Studies at the National

Institutes of Health in Bethesda, Md. -- has been

relatively inactive for the past several years and

will come to a complete halt at the end of the week.

That is when the last remaining member, Dr. Paul

Brown, will retire after 40 years of dealing with

these mysterious and fatal diseases, known

collectively as transmissible spongiform

encephalopathies.

 

TSEs, which include mad cow disease and its human

equivalent, Creutzfeldt Jakob disease, destroy the

brain and are agonizing for both the unlucky victims

and their family members who can only stand by

helplessly as loved ones are consumed by the incurable

disorder.

 

The NIH's decision to shut down the lab is confusing

to some experts in the field -- including Brown --

particularly so because these diseases are not fully

understood and there is no therapy or cure in sight.

 

" It's been difficult for me to understand, " said

Brown, who serves as medical director of the CNSS lab.

" They're (the NIH administration) simply going in a

different direction and want to spend their money

elsewhere and they just, for inexplicable reasons, are

not interested in promulgating CJD in-house research, "

he told UPI.

 

" CJD simply comes under the umbrella of preferring not

to deal with it, " Brown said, emphasizing that his

comments were not meant to be considered sour grapes

or criticism. " I'm much surprised, viewed in the

interest and importance of this group of diseases,

that NIH would choose to go in the direction they've

gone, but it is their decision to make. "

 

A panel of 12 experts assembled by the Institute of

Medicine agreed with Brown on the importance of

keeping an in-house TSE lab operating at the NIH. The

panel recommended in a report released late last year

(but not officially published until April 2004) that

the NIH re-establish a lab similar to the LCNSS,

because it would aid in the development of diagnostics

and drugs to treat the TSEs, which many scientists

think are caused by abnormal proteins called prions.

 

The institute's 288-page report, " Advancing Prion

Science: Guidance for the National Prion Research

Program, " notes that " the infrastructure for TSE

research in the United States is small, aging, and

funded at a level below that needed to achieve the

research goals ... expeditiously. " It adds, " Efforts

to re-establish an intramural NIH laboratory on human

prion diseases should be encouraged, " because " it

could better handle issues of infrastructure

investment, sustained funding, and investigator

security than extramural program in universities and

private institutes. "

 

Eugene Major, acting director of the basic

neuroscience program at the NIH, told UPI the agency

is considering the possibility of re-establishing

another lab like CNSS, but at present they think they

can achieve their TSE research goals by funding

outside laboratories at universities.

 

" I don't think we're losing anything, " Major told UPI.

" The institute continues to support and consider prion

science a very important area, " he added.

 

The NIH supports TSE research internally at the Rocky

Mountain Lab in Hamilton, Mont., but this lab tends to

focus on the animal TSEs, such as scrapie in sheep and

chronic wasting disease in deer and elk, rather than

the human forms.

 

The CNSS lab was instrumental in the 1960's in showing

a rare and fatal brain disease known as kuru that

occurred in the Fore people in New Guinea was

infectious. The work won the lab's first director,

Carleton Gajdusek, a Nobel prize in 1976 and helped

pave the way for discovering that prions are involved

in causing TSEs.

 

Brown's research helped demonstrate the nearly

indestructible nature of prions. In one controversial

experiment, he took a sample of prion-containing

tissue home with him and buried it in a flower pot in

his backyard. The agent was still infectious when he

dug it up three years later.

 

In another study, Brown showed prions could remain

infectious even after going through temperatures as

high as 1,110 degrees Fahrenheit (600 degrees

Celsius). On the preventative side, Brown developed an

ultra-high pressure method of inactivating prions in

processed meat, which could help ensure it is safe

from contamination with mad cow disease, but this

technique has not yet been employed by meat companies.

 

At the same time the NIH is moving to shut down the

CNSS lab, new discoveries about TSEs are being made in

other parts of the world.

 

Although it appeared the outbreak of variant CJD --

the form of CJD humans can contract by consuming meat

infected with the mad cow pathogen -- was declining in

the United Kingdom, a recent study indicated as many

as 3,800 people may be unwittingly infected, but not

yet showing symptoms. It also was reported recently

that a second person had acquired vCJD through a blood

transfusion, raising concerns about how many

additional people worldwide received infected blood

and might be at risk of having contracted the disease.

 

TSEs also are presenting problems in the United

States. The first confirmed case of mad cow disease

was detected in Washington state last December and

U.S. Department of Agriculture officials have said

they expect to find additional cases as they ramp up

the number of cows tested for the disorder. Also,

chronic wasting disease is spreading in deer and elk

in the midwestern United States and Canada and

researchers are concerned it could infect humans

--although no confirmed cases have been detected so

far.

 

Dr. Richard Johnson, a neurologist at the Johns

Hopkins University School of Medicine in Baltimore and

chair of the committee that developed the

recommendations in the Institute of Medicine report,

said the time-limited grants from the NIH -- they

generally cover three-year increments -- and the

limited funding available for TSE research is " a very

strong disincentive for people to go into this work, "

because prion research is expensive and it can take

several years to run one experiment due to the long

incubation time of these diseases.

 

" The solution for that would be for the government to

establish a lab within NIH that would concentrate on

human diseases, " similar to the CNSS lab, Johnson told

UPI. He noted the Centers for Disease Control and

Prevention's Rocky Mountain lab in Denver is not a

suitable surrogate, because it traditionally has

concentrated on the animal forms of these diseases.

 

Dr. Bruce Chesebro, chief of the Laboratory of

Persistent Viral Diseases at the Rocky Mountain lab,

said he agrees " completely " with the recommendations

in the institute's report for maintaining an in-house

lab at the NIH headquarters in Bethesda, Md.

 

This would help foster clinical research for possible

treatments, Chesebro said. The Rocky Mountain lab is

interested in the human side of these diseases but

they don't have a hospital, so a facility in Bethesda,

where there is a hospital and clinical research

experts, would help encourage that type of research,

he said.

 

At the moment, that type of research is sorely needed.

An NIH database of ongoing clinical trials for

experimental therapies does not contain a single study

for CJD, an indication there is no promising therapy

on the horizon for this fatal disease.

 

--

 

Steve Mitchell is UPI's Medical Correspondent. E-mail

sciencemail

 

2001-2004 United Press International

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