Navigation Links
Model found to determine cause, possible treatment of nerve tumors
Date:2/4/2008

CINCINNATI A multi-institutional study led by researchers at Cincinnati Childrens 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 Childrens. 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 Childrens has a leading multi-disciplinary clinic for diagnosis and management of NF1.


'/>"/>

Contact: Nick Miller
513-803-6035
Cincinnati Children's Hospital Medical Center
Source:Eurekalert

Related medicine news :

1. 32-Year-Old Breast Cancer Survivor, Fashion Model Featured in Benefit Concert Music of the Light at Skirball Cultural Center
2. Mouse Model of Age-Related Macular Degeneration Developed
3. In silico modeling helps predict severity of mitochondrial disease
4. Researchers train the immune system to deliver virus that destroys cancer in lab models
5. Transtheoretical model may help to identify OSA patients level of intention to exercise
6. Researchers build new model of bio-exploration
7. Supermodel Rachel Hunter Helps Over 300 People Find Their Slim
8. Modeling Simulation Predicts Potential Negative Impact on U.S. Blood Supply if ESA Use Limited for Chemotherapy-Induced Anemia
9. EntreMed Presents Results for ENMD-1198 and Vincristine in Preclinical Leukemia Models
10. SARS: a model disease
11. Consumer Group to Nunez: Massachusetts Mandatory Health Insurance Purchase Law Is No Model for California
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:2/14/2016)... Laurel, NJ (PRWEB) , ... February 14, 2016 ... ... 14-20, 2016, the American Association of Heart Failure Nurses (AAHFN) is promoting healthier ... caregivers. This year’s campaign, Spices of Life! Healthy Living with Heart Failure will ...
(Date:2/13/2016)... ... February 13, 2016 , ... Many individuals looking to lead a healthy lifestyle ... reasons. IsoPasta by Isolator Fitness has delved into this niche allowing those giving ... high-carb repercussions. IsoPasta has 30 grams of protein and only 7 grams of ...
(Date:2/13/2016)... Salt Lake City, Utah (PRWEB) , ... February 13, 2016 , ... When an ... kids, Host Parents aren’t always sure what they are in for and they are often ... Pairs are more than they were hoping for. This year’s Au Pair of the Year ...
(Date:2/12/2016)... ... February 12, 2016 , ... The law firm of Morrow, Morrow, Ryan & ... purpose of these scholarships is to encourage applicants to pursue a degree in their ... these two parishes. , “We have available jobs in St. Landry and Evangeline ...
(Date:2/12/2016)... (PRWEB) , ... February 12, 2016 , ... Each year, ... will be held in Anaheim, CA at the Anaheim Convention Center. Almost 10,000 physical ... see new therapy products in action, learn more about their chosen field and network ...
Breaking Medicine News(10 mins):
(Date:2/12/2016)... 12 2016  OMS Supply, a large provider of ... announced today the recent launching of their new company ... of features that enhance the user experience and enable ... --> --> Despite ... company that started in early 2016, they have already ...
(Date:2/12/2016)... 12, 2016 Indiso ... den ungedeckten medizinischen Bedarf bei Lungen- und ... klinischen Forschungsprogramms bekannt. Das Programm, das sich ... ihrer respiratorischen Funktionen und anderer klinischer Parameter. ... Medizintechnikunternehmen, das sich auf den ungedeckten medizinischen ...
(Date:2/11/2016)... The primary goal of this research is to analyze ... of liquid biopsy. Key information the survey seeks to ... liquid biopsy adoption amidst future users - Predominantly used ... Sample inflow to conduct liquid biopsy tests - Preference ... on. - Correlation analysis of sample type and biomarker ...
Breaking Medicine Technology: