Featured Research

from universities, journals, and other organizations

Model Found To Determine Cause, Possible Treatment Of Nerve Tumors

Date:
February 7, 2008
Source:
Cincinnati Children's Hospital Medical Center
Summary:
A new model may help unravel the cause of fibrous, noncancerous nerve tumors called neurofibromas, which can lead to disfigurement and in rare cases death by compressing vital organs. Researchers said their findings also provide a way to test therapies that could eventually help patients with limited options for treating the disease -- Neurofibromatosis type 1.

A multi-institutional study led by researchers at Cincinnati Children's Hospital Medical Center has led to new insights and a model to help unravel the cause of fibrous, non-cancerous nerve tumors called neurofibromas, which can lead to disfigurement and in rare cases death by compressing vital organs. Writing for the February Cancer Cell, researchers said their findings also provide a means for testing therapies that could eventually help patients who now have limited options for treating the disease, called Neurofibromatosis type 1.

"We have developed a mouse model that allows us to mimic as closely as possible the formation in humans of two kinds of neurofibromas, which are non-cancerous tumors that affect children and adults," said Nancy Ratner, Ph.D., study co-author and a researcher in the Division of Experimental Hematology and Cancer Biology at Cincinnati Children's. "Our findings give us a way to begin clarifying the biological mechanisms underlying the tumor formation in Neurofibromatosis and to have a platform for therapeutic testing."

Previous research has determined that neurofibromas result from a mutation in a gene (called Nf1), which normally acts as a tumor suppressor by producing a complex protein called neurofibromin to help regulate cell growth. Mutation of the Nf1 gene inhibits neurofibromin production, leading to irregular cell growth and possible tumor development. Dr. Ratner and her colleagues suggest in their study that the timing of this gene mutation is critical to whether neurofibroma tumors form.

In one set of experiments, the researchers worked with genetically engineered nerve root cells from embryonic mice. They discovered that mutating both of two mouse Nf1 genes at 12.5 days after conception (a critical stage in mouse embryonic development when cells that eventually differentiate to form the nervous system are just getting ready to do so) would create cell colonies. In another set of experiments, researchers worked with mice that were genetically engineered for expression of a gene called Desert Hedgehog (Dhh) using a genetic trick to cause Nf1 mutation only in cells that express that gene.

Again, the researchers discovered that by mutating both Nf1 genes at 12.5 days post conception in developing nerve cells, the resulting adult mice developed neurofibromas and had shorter life spans than their littermates whose genes were not mutated. If the mutations occurred earlier or later than 12.5 days, in both sets of experiments neurofibroma cells did not begin to form.

Dr. Ratner and her colleagues report that because nerves from patients with Neurofibromatosis type 1 have not been analyzed, direct comparisons between disease formation in humans and mice can not yet be made.

Neurofibromatosis 1 is an autosomal dominant inherited disease, which means the gene mutation only needs to occur in one chromosome for a person to get the disease. Disease symptoms may be evident at birth, during infancy and almost always by the time a child is 10 years old. The condition affects one of every 3,000 people worldwide with nearly 90 percent of the patients developing neurofibromas. Many NF1 patients conduct normal lives with the disease, with the primary manifestation being numerous soft brown pigment spots on the skin, and numerous tumors that are very small under the skin. Other patients, however, develop learning disabilities, scoliosis, and benign brain tumors as children, and are at risk for cardiovascular problems and malignant tumors as adults. Cincinnati Children's has a leading multi-disciplinary clinic for diagnosis and management of NF1.

The study included researchers from the Hoxworth Blood Center, University of Cincinnati College of Medicine; Departments of Pathology at Massachusetts General Hospital and Harvard Medical School (Boston) and the Departments of Cell Biology and Genetics at Erasmus University Medical Center (Rotterdam, Netherlands). Also offering assistance through provision of genetically engineered mice and other materials were the University of Texas Southwestern, the Stowers Institute and Rockefeller University. Funding support came from the National Institutes of Health.


Story Source:

The above story is based on materials provided by Cincinnati Children's Hospital Medical Center. Note: Materials may be edited for content and length.


Cite This Page:

Cincinnati Children's Hospital Medical Center. "Model Found To Determine Cause, Possible Treatment Of Nerve Tumors." ScienceDaily. ScienceDaily, 7 February 2008. <www.sciencedaily.com/releases/2008/02/080204143153.htm>.
Cincinnati Children's Hospital Medical Center. (2008, February 7). Model Found To Determine Cause, Possible Treatment Of Nerve Tumors. ScienceDaily. Retrieved July 28, 2014 from www.sciencedaily.com/releases/2008/02/080204143153.htm
Cincinnati Children's Hospital Medical Center. "Model Found To Determine Cause, Possible Treatment Of Nerve Tumors." ScienceDaily. www.sciencedaily.com/releases/2008/02/080204143153.htm (accessed July 28, 2014).

Share This




More Health & Medicine News

Monday, July 28, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

$15B Deal on Vets' Health Care Reached

$15B Deal on Vets' Health Care Reached

AP (July 28, 2014) A bipartisan deal to improve veterans health care would authorize at least $15 billion in emergency spending to fix a veterans program scandalized by long patient wait times and falsified records. (July 28) Video provided by AP
Powered by NewsLook.com
Traditional African Dishes Teach Healthy Eating

Traditional African Dishes Teach Healthy Eating

AP (July 28, 2014) Classes are being offered nationwide to encourage African Americans to learn about cooking fresh foods based on traditional African cuisine. The program is trying to combat obesity, heart disease and other ailments often linked to diet. (July 28) Video provided by AP
Powered by NewsLook.com
West Africa Gripped by Deadly Ebola Outbreak

West Africa Gripped by Deadly Ebola Outbreak

AFP (July 28, 2014) The worst-ever outbreak of the deadly Ebola epidemic grips west Africa, killing hundreds. Duration: 00:48 Video provided by AFP
Powered by NewsLook.com
Trees Could Save More Than 850 Lives Each Year

Trees Could Save More Than 850 Lives Each Year

Newsy (July 27, 2014) A national study conducted by the USDA Forest Service found that trees collectively save more than 850 lives on an annual basis. Video provided by Newsy
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:

Breaking News:
from the past week

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile: iPhone Android Web
Follow: Facebook Twitter Google+
Subscribe: RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins