Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

MGH Cancer Center researchers find new gene associated with Wilms tumor

09.01.2007
Mutations to gene on X chromosome found in 30 percent of pediatric kidney cancer cases

Researchers at the Massachusetts General Hospital (MGH) Cancer Center have discovered a novel gene mutation associated with Wilms tumor, the most common pediatric kidney cancer. The newly identified gene is mutated in about 30 percent of cases of Wilms tumor and is located on the sex-determining X chromosome, which means that a single altered copy would be sufficient for tumor formation. The new gene does not appear linked to inherited forms of the disease.

"This is the first X chromosome gene directly implicated as a tumor suppressor," says Daniel A. Haber, MD, PhD, director of the MGH Cancer Center and senior author of the report, which will appear in the journal Science and is receiving early online release on the Science Express website at http://www.sciencexpress.org. "It has the potential of someday being a useful prognostic marker for Wilms tumor patients, and learning about its normal function could tell us more about both normal kidney development and tumorigenesis."

Also called nephroblastoma, Wilms tumor develops in one out of 10,000 children and is usually treated successfully with surgery and chemotherapy. Mutations in a gene called WT1, first identified in 1990, cause about 5 percent of cases, and a few other genes are associated with rare syndromes that can include Wilms tumor. Those with a family history of the disease have an increased risk of developing the cancer in both kidneys.

... more about:
»Haber »Kidney »MGH »Pediatric »WTX »X chromosome

Since so few cases of Wilms tumor could be attributed to the identified mutations, the MGH Cancer Center researchers analyzed tumor samples from 82 patients to search for additional genetic abnormalities. Genome screening and sequencing tests showed that nearly 30 percent of the samples had either deletions or mutations in the same area of the X chromosome, indicating a new cancer gene that the researchers have named WTX. In samples from female patients, mutated copies of WTX were found only on the active copy of the X chromosome.

"Males have only one X chromosome, so for them a single mutation can silence the gene and cause a tumor," Haber explains. "Females have two X chromosomes, but one is inactivated during normal development. We showed that mutations specifically occur on the active X in female Wilms patients, so it takes a single genetic event to inactivate WTX in either males or females. That's in contrast to other tumor suppressor genes, which only can be inactivated by independent mutations affecting both copies of the gene."

The researchers also found that WTX is expressed in cells involved in embryonic kidney development, suggesting that it normally plays a key role in the organ's formation. They are now investigating the gene's normal function and studying its disruption in an animal model.

"The biology that links pediatric cancers to normal organ development is fascinating," says Haber. "Adult kidney cancers arise slowly from the organ's tubules and are highly resistant to current chemotherapy drugs, but pediatric kidney tumors arise in the early stem cells of the kidney's filtering apparatus and are highly responsive to chemotherapy. Following up on these findings should help us better understand this tumor and may lead to a new appreciation of the X chromosome's role in other forms of cancer." Haber is the Laurel Schwartz Professor of Oncology at Harvard Medical School.

Sue McGreevey | EurekAlert!
Further information:
http://www.mgh.harvard.edu/

Further reports about: Haber Kidney MGH Pediatric WTX X chromosome

More articles from Life Sciences:

nachricht Plant escape from waterlogging
17.10.2017 | Christian-Albrechts-Universität zu Kiel

nachricht Study suggests oysters offer hot spot for reducing nutrient pollution
17.10.2017 | Virginia Institute of Marine Science

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

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

Im Focus: Breaking: the first light from two neutron stars merging

Seven new papers describe the first-ever detection of light from a gravitational wave source. The event, caused by two neutron stars colliding and merging together, was dubbed GW170817 because it sent ripples through space-time that reached Earth on 2017 August 17. Around the world, hundreds of excited astronomers mobilized quickly and were able to observe the event using numerous telescopes, providing a wealth of new data.

Previous detections of gravitational waves have all involved the merger of two black holes, a feat that won the 2017 Nobel Prize in Physics earlier this month....

Im Focus: Smart sensors for efficient processes

Material defects in end products can quickly result in failures in many areas of industry, and have a massive impact on the safe use of their products. This is why, in the field of quality assurance, intelligent, nondestructive sensor systems play a key role. They allow testing components and parts in a rapid and cost-efficient manner without destroying the actual product or changing its surface. Experts from the Fraunhofer IZFP in Saarbrücken will be presenting two exhibits at the Blechexpo in Stuttgart from 7–10 November 2017 that allow fast, reliable, and automated characterization of materials and detection of defects (Hall 5, Booth 5306).

When quality testing uses time-consuming destructive test methods, it can result in enormous costs due to damaging or destroying the products. And given that...

Im Focus: Cold molecules on collision course

Using a new cooling technique MPQ scientists succeed at observing collisions in a dense beam of cold and slow dipolar molecules.

How do chemical reactions proceed at extremely low temperatures? The answer requires the investigation of molecular samples that are cold, dense, and slow at...

Im Focus: Shrinking the proton again!

Scientists from the Max Planck Institute of Quantum Optics, using high precision laser spectroscopy of atomic hydrogen, confirm the surprisingly small value of the proton radius determined from muonic hydrogen.

It was one of the breakthroughs of the year 2010: Laser spectroscopy of muonic hydrogen resulted in a value for the proton charge radius that was significantly...

Im Focus: New nanomaterial can extract hydrogen fuel from seawater

Hybrid material converts more sunlight and can weather seawater's harsh conditions

It's possible to produce hydrogen to power fuel cells by extracting the gas from seawater, but the electricity required to do it makes the process costly. UCF...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

ASEAN Member States discuss the future role of renewable energy

17.10.2017 | Event News

World Health Summit 2017: International experts set the course for the future of Global Health

10.10.2017 | Event News

Climate Engineering Conference 2017 Opens in Berlin

10.10.2017 | Event News

 
Latest News

Study suggests oysters offer hot spot for reducing nutrient pollution

17.10.2017 | Life Sciences

Breaking: the first light from two neutron stars merging

17.10.2017 | Physics and Astronomy

World first for reading digitally encoded synthetic molecules

17.10.2017 | Life Sciences

VideoLinks
B2B-VideoLinks
More VideoLinks >>>