British Research Breakthrough Brings Hope To Neuroblastoma Children

Main Category: Cancer / Oncology
Also Included In: Pediatrics / Children's Health
Article Date: 24 Apr 2009 - 8:00 PDT

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A gene first discovered 20 years ago has been identified as a key to preventing one of the biggest cancer killers of children.

The common gene clusterin, also known as apolipoprotein J, is present in most human tissues and body fluids.

Now researcher Dr Arturo Sala, of London's internationally-renowned UCL Institute of Child Health, has established the first scientific evidence that clusterin is a tumour suppressor of neuroblastoma.

Neuroblastoma is the most common extra-cranial solid tumour in childhood and 90% of cases occur in children Under 5. It is one of the 3 biggest causes of cancer deaths among children and the biggest cause of cancer fatalities among babies.

Dr Sala's groundbreaking research which was funded by the British children's medical research charity Sparks, has just been published in America in the prestigious Journal of the National Cancer Institute.

Explains Dr Sala: "Our team found that in neuroblastoma expression of clusterin is repressed by the cancer gene MYCN, which is typically activated in this childhood malignancy.

"When expression of clusterin was artificially restored in cultured neuroblastoma cells, these were not malignant and could not form tumours during laboratory experiments. This strongly suggests that clusterin can effectively suppress tumour growth."

Dr Sala's team are now investigating whether the drugs that cause reactivation of clusterin expression in aggressive neuroblastomas can stop the growth of the cancer, giving new hopes to children affected by this terrible disease.

Aggressive neuroblatoma affects between 80 and 100 children a year in the UK, with a fatality level of more than 50%. It also fails to respond as well to the intensive treatments that have reduced fatality rates among other childhood cancers, including leukaemia.

Dr Sala added: " Neuroblastoma is rarer and much less well-known than leukaemia despite the very high rate of fatalities and research is very poorly funded by comparison. That's why the efforts being made in this field by Sparks are so very important."

Dr Sala, who hopes to be able to start human clinical trials in about 3 years, believes that his clusterin breakthrough could also prove a key in tackling other cancers, including prostate cancer.

Sparks, the children's medical research charity which has already invested over £1.5million in neuroblastoma research, recently launched 'George's Appeal' in a bid to raise a further £2m to try and beat this particularly vicious childhood-only cancer killer. It's named after 9-year George Yeomans, who lost his brave fight against the disease last year after helping Sparks raise awareness of the desperate need for more research funding.

Paul Connew, Sparks Director of Communications, said: "Dr Sala's research work is clearly of great importance and could lead to major improvements in the prospects of children with neuroblastoma. This is a crucial area of childhood cancer research and Sparks is committed to raising more funding to support vital research work by Dr Sala and others in the neuroblastoma field."

Dr John Anderson, another of Britain's leading neuroblastoma experts who is also funded by Sparks is also running this years London Marathon to raise awareness and support for research into neuroblastoma and George's appeal.

The appeal campaign is being actively supported by a number of Sparks celebrity Ambassadors, including president Gabby Logan, Martin Johnson and Ronnie Corbett.

Actors Chris Coghill and Ryan Philpott, of Eastenders, Hollyoaks star Mikyla Dodd, together with Olympic badminton heroine Gail Emms and former Leicester Tigers rugby star Derek Jelley , ran the London Marathon in memory of George and to support the fundraising effort.

John Altman (EastEnders 'Nasty Nick' Cotton) and Dean Andrews, of 'Ashes to Ashes' and Life on Mars' fame are running this summer's Leeds 10k to support the appeal.

Source
British Research Breakthrough

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