[HEADLINE: Cure hope for athletes' heart killer]

Author: Tim Stewart (67.39.31.---)

Date: 11-18-02 06:06

Copyright 2002 Times Newspapers Limited

Sunday Times (London)

November 10, 2002, Sunday

SECTION: Home news; Scotland News 8

LENGTH: 812 words

HEADLINE: Cure hope for athletes' heart killer

BYLINE: Sue Leonard

BODY:

DOCTORS have discovered the cause of sudden death syndrome (SDS), a

condition that has killed a number of young athletes.

The breakthrough by Scottish scientists could lead to the first effective

treatment for the heart condition - also known as hypertrophic cardiomyopathy

(HCM) - which affects 100,000 people in the UK and kills about 200 every year.

It is the main cause of sudden death in young athletes and has claimed the

lives of Daniel Yorath, the brother of Gabby Logan, the television presenter,

Andy Thomson, the Motherwell football player, and Tony Penny, the basketball

player.

Chris Proud, professor of biochemical physiology at Dundee University, has

discovered the biological process which leads to a build-up of protein around

the heart, causing the muscle to thicken so much that it leads to heart failure.

Proud found that in HCM, a key protein called MAP kinase is "switched on"

too much by hormones which act on the heart. In hypertrophy the body responds to

the load on the heart by making more of particular hormones which switch on MAP

kinase - this in turn makes the cells produce more protein and causes the heart

to grow abnormally large.

As the heart muscle grows, the heart's contractions start to malfunction and

it becomes less able to pump blood to the rest of the body.

Working on animal heart cells, Proud used a drug that is being developed for

the treatment of cancer to switch off the protein, leading to hopes that a

treatment could now be developed for HCM sufferers.

"We have found a way of preventing the disease that leads to sudden death in

athletes," he said last week.

"We could potentially design drugs to tackle the disease and treat it. If

you can prevent this protein from being turned on inappropriately, then you

could block the process of overgrowth of the heart.

"It is an important discovery for thinking about future therapies to prevent

this condition or potentially to reverse it."

Although the condition is thought to be genetic in at least 70% of cases,

many people do not even know that they suffer from it.

Daniel Yorath was just 15 years old and had been signed by Leeds United when

he suddenly collapsed after playing football in his parents' garden. In 1998

Thomson collapsed and died of SDS while celebrating his 19th birthday.

Other sporting victims include David Longhurst, a 25-year-old York City

player who collapsed and died during a televised match; John Marshall, a

16-year-old junior international footballer of the year who died on the day that

he was due to sign for Everton; Laura Moss, 13, a swimmer, and Adrian Hawkins,

22, who was shortlisted for a place in the Great Britain cycling team at the

Barcelona Olympics when he died after a race.

Those known to have a family history of the disease could in future be

screened and treated as soon as any abnormality is found so as to prevent damage

to the heart.

There are a number of treatments available at present to tackle the symptoms

of HCM once the disease has developed and to prevent complications.

However, those diagnosed have to give up their sport and are treated with

drugs such as beta-blockers. Some people may require surgery or even a heart

transplant.

Proud said: " At the moment people are tackling the consequences. It is

better to prevent it from happening in the first place. It is more difficult to

put right a defective heart than it is to prevent it becoming defective in the

first place."

Proud says a therapy could be available in five to 10 years.

"We have discovered some of the underlying events that cause the heart to

get bigger, and because a lot is known about this process it is possible to

target it with drugs," he said.

"You can't tackle the hormones at the root of this so easily, but you can

prevent the hormones having this undesirable effect on the production of protein

in the heart. You can turn the switch off again."

The results of the four-year research, funded by the British Heart

Foundation, have just been published in the journal Circulation Research.

A Cardiomyopathy Association spokeswoman said: "The CMA welcomes any

developments that will assist people with cardiomyopathy. All too many lives are

still being lost because of the condition."

o Scientists in Glasgow have pinpointed the location of a gene that may be

crucial for ensuring that cells die when they should, giving hope of

developments in the treatments for cancers affecting the liver, throat, lung and

cervix.

Researchers at the Cancer Research UK Beatson laboratories have narrowed

down the search for the gene to a tiny section of chromosome four - one of the

bundles of DNA containing our genes.

They believe that the gene may form part of the internal controls that in

healthy tissue prevent cells from living and dividing indefinitely.

LOAD-DATE: November 15, 2002

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[Re: HEADLINE: Cure hope for athletes' heart killer]

Author: Dee (---.jvl.wi.charter.com)

Date: 11-18-02 12:40

I'm just wondering if I'm the only one that got really excited when I read this article. (this is my first time posting by the way - haven't had a chance to introduce myself and my son who has HOCM).

I'm REALLY excited after reading this article. Is this correct and reputable, etc??

Dee

(Mom to Dylan, 3 years old, HOCM, Stroke/Endocarditis)

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[Re: HEADLINE: Cure hope for athletes' heart killer]

Author: karen (---.dyn.optonline.net)

Date: 11-18-02 14:38

Hi Dee - Mom to Dylan (I love that name!)

I found this article interesting, so much so that I emailed it to my children's cardiologist. I was happy to read it, but am skeptical. I'm under the impression that the sloppy, unorganized myocardial cells plays a part in the sudden death issue along with the muscle thickness.

This is good, it is certainly a start, but I can't say I was really excited Dee.

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[Re: HEADLINE: Cure hope for athletes' heart killer]

Author: Tom (---.eosinc.com)

Date: 11-18-02 17:22

The way I see it, it's a research doctor who is optimistic about future treatment and potential prevention of HCM. If he's optimistic, so am I. It sounds like he's on the right track and I hope he continues to push hard after this.

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[Re: HEADLINE: Cure hope for athletes' heart killer]

Author: Lisa Salberg (167.165.39.---)

Date: 11-18-02 19:39

Hold the party for a little while guys... This is Lisa with a reality check (sorry). This is unproven, it is in animals not people AND this does not stop the gene...it may repeat may limit hypertophy... so lets see the data, lets have the medical prof. weigh in and lets see what we have here.

this is not a disease of hypertrophy...it is a disease of abnormal cell structure...this is a disease that is in the very fiber of our being, it would be wonderful if we could 'turn off the gene' but we would have to know all the genes 1st...

ALSO.. data from the Siedman lab in Boston has shown that if you give some medications to mice (little geneticly altered HCM mice)you can reduce the amount of hypertrophy...this data has been around for about a year or so therefore I am not too suprised by the claims made by the group in Scottland.

I wish the researchers well in there work and hope they are headed in the right direction. but no party yet...soon I hope...but I really think we are 10+ years away from anything BIG.

All the best!

Lisa