Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:


Pediatric cancer : Alteration of a gene causes neuroblastoma

Olivier Delattre’s team (Inserm Unit 830 “Genetics and Biology of Cancer”) of the Institut Curie reveal in an article in the 16 October issue of Nature that alteration of the ALK gene is closely associated with the most frequent solid extracerebral tumor in children—neuroblastoma.

By studying the familial forms of this tumor, the researchers also conclude that ALK is a gene that predisposes to neuroblastoma. This discovery may allow the development of new treatments in neuroblastomas.

It may also enable the identification in at-risk families of children who carry an altered ALK gene, so that they can be offered regular checkups. At present, over half of children diagnosed with neuroblastoma and metastases still die of their disease.

Neuroblastoma is the most frequent solid extracerebral tumor in young children. Some 50% of children affected—boys and girls alike—are under two years of age. In France, there are about 150 new cases every year, and one quarter of these are treated at the Institut Curie, a reference center recognized worldwide for its neuroblastoma research and treatment.

Neuroblastoma develops in the sympathetic nervous system, more specifically in the small round cells derived from the neural crest, a transient region characteristic of vertebrate embryos. It principally affects the abdomen, and more rarely the thorax, neck, and lower pelvis.

ALK, a key participant in development of neuroblastoma

At the Institut Curie, Olivier Delattre, Director of Inserm Unit 830 “Genetics and Biology of Cancer”, and his team have just shown that the ALK gene is involved right from the earliest stages of neuroblastoma development.

Through a national collaboration with other centers that treat neuroblastoma, and exemplary exchanges between physicians and researchers at the Institut Curie, Olivier Delattre and his group “genetically” analyzed 592 neuroblastoma samples. They found that in these tumors the ALK gene was frequently altered: either there were excess copies or it contained activating mutations. Now, inhibition of the ALK gene in neuroblastoma cells greatly reduces cell proliferation.

The researchers have also shown that in the familial forms of neuroblastoma, mutations of the ALK gene are transmitted to offspring. ALK thus acquires the status of neuroblastoma predisposition gene. In a study of ten familial forms, an American team confirms this result in the same issue of Nature (Y.P. Mosse, et al. Nature, doi:10.103).

This discovery should also lead to an improved clinical management and follow-up of at risk patients within neuroblastoma families with ALK mutations. The tight links between the Inserm U830 and Institut Curie Genetics Department, directed by Prof Dominique Stoppa-Lyonnet, should constitute an asset for these clinical applications;
Therapeutic applications should soon follow from this discovery, since as Olivier Delattre points out the “ALK gene is also involved in certain lymphomas and one type of lung cancer, and drugs that can counteract its effect have already been identified.” ALK encodes a tyrosine kinase receptor on the cell surface which acts as a switch. Permanently switched on in tumor cells, it tells them to multiply ceaselessly. Dr Delattre, who is both a researcher and pediatrician, observes that “in other cancers, as for instance breast cancer, drugs able to block the effect of this type of receptor—Herceptin® to name but one—are already used routinely.” Preclinical studies should soon be under way to assess whether these drugs can stop on neuroblastoma growth. This holds out great hope to young patients since, in certain forms, currently available chemotherapy fails to stop rapid progression of the neuroblastoma.

This study was funded by the Ligue contre le cancer, the Association des Parents et des Amis des Enfants Soignés à l’Institut Curie (APAESIC), Bagouz à Manon, Association Hubert Gouin, and Les Amis de Claire et Enfants et Santé.

Céline Giustranti | alfa
Further information:

More articles from Life Sciences:

nachricht Molecular doorstop could be key to new tuberculosis drugs
20.03.2018 | Rockefeller University

nachricht Modified biomaterials self-assemble on temperature cues
20.03.2018 | Duke University

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

Die letzten 5 Focus-News des innovations-reports im Überblick:

Im Focus: Mars' oceans formed early, possibly aided by massive volcanic eruptions

Oceans formed before Tharsis and evolved together, shaping climate history of Mars

A new scenario seeking to explain how Mars' putative oceans came and went over the last 4 billion years implies that the oceans formed several hundred million...

Im Focus: Tiny implants for cells are functional in vivo

For the first time, an interdisciplinary team from the University of Basel has succeeded in integrating artificial organelles into the cells of live zebrafish embryos. This innovative approach using artificial organelles as cellular implants offers new potential in treating a range of diseases, as the authors report in an article published in Nature Communications.

In the cells of higher organisms, organelles such as the nucleus or mitochondria perform a range of complex functions necessary for life. In the networks of...

Im Focus: Locomotion control with photopigments

Researchers from Göttingen University discover additional function of opsins

Animal photoreceptors capture light with photopigments. Researchers from the University of Göttingen have now discovered that these photopigments fulfill an...

Im Focus: Surveying the Arctic: Tracking down carbon particles

Researchers embark on aerial campaign over Northeast Greenland

On 15 March, the AWI research aeroplane Polar 5 will depart for Greenland. Concentrating on the furthest northeast region of the island, an international team...

Im Focus: Unique Insights into the Antarctic Ice Shelf System

Data collected on ocean-ice interactions in the little-researched regions of the far south

The world’s second-largest ice shelf was the destination for a Polarstern expedition that ended in Punta Arenas, Chile on 14th March 2018. Oceanographers from...

All Focus news of the innovation-report >>>



Industry & Economy
Event News

Virtual reality conference comes to Reutlingen

19.03.2018 | Event News

Ultrafast Wireless and Chip Design at the DATE Conference in Dresden

16.03.2018 | Event News

International Tinnitus Conference of the Tinnitus Research Initiative in Regensburg

13.03.2018 | Event News

Latest News

Physicists made crystal lattice from polaritons

20.03.2018 | Physics and Astronomy

Mars' oceans formed early, possibly aided by massive volcanic eruptions

20.03.2018 | Physics and Astronomy

Thawing permafrost produces more methane than expected

20.03.2018 | Earth Sciences

Science & Research
Overview of more VideoLinks >>>