JUL 16, 2017 10:00 AM PDT

Research on Neurological Tumor Development Reveals Drug Target

WRITTEN BY: Carmen Leitch

Scientists have learned more about the role of a molecule called prion protein (PrPC) in the development of some types of neurological tumors. This work could have a huge impact on the treatment of several cancerous diseases, like breast cancer, melanoma, glioblastoma, mesothelioma, and neurofibromatosis 2 (NF2), a hereditary genetic disorder carried by thousands of people.

This is a schwannoma tumor cell. / Credit: University of Plymouth

NF2 causes tumor growth on the nervous system. These tumors - ependymomas, schwannomas, and meningiomas - have been associated with mutations in the tumor suppressor gene Merlin. The researchers discovered that a deficiency of Merlin is linked to the overproduction of PrPC in schwannoma compared with healthy Schwann cells. This work, by investigators at the University of Plymouth and Plymouth Hospitals NHS Trust, has been reported in Oncogene.

Normally, the PrPC protein is important during embryonic development and acts in neuroprotection in healthy adults. However, it is absent in patients with Creutzfeldt-Jakob prion disease that carry a deleterious variant of a prion protein - scrapie prion protein (PrPSc). There are elevated levels of PrPC in several cancers like breast, gastric and prostate cancer and glioblastoma.

The scientists have also identified a number of existing drugs that might be useful for reducing protein production. Because these drugs have been approved already for the treatment of Acute Myeloid Leukemia (AML), Creutzfeldt-Jakob disease, and multiple myeloma, it could be relatively straightforward to orchestrate clinical studies for repurposing them.

"By understanding the relationship between overproduction of PrPC and Merlin deficiency in the development of schwannoma and meningioma, we have made a significant stride forward in the search for a drug treatment for NF2. This is a life-changing condition usually striking the young. That our discovery could also lead to hope for thousands of patients affected by other Merlin-deficient tumors, adds yet more to the significance and excitement of our findings,” commented Dr. Sylwia Ammoun, Senior Research Fellow in Clinical Neurobiology.

"Identifying a range of existing drugs which could be repurposed is an exciting approach by which we can speed up the progress of scientific discovery from the lab to the clinic, without compromising safety. Brain tumors kill more children and adults under the age of 40 than any other cancer, but a lack of research funding over decades has meant that current treatments lag well behind those of leukemia and many other cancers. We are having to play catch up to improve outcomes for patients and this study will bring us closer to identifying new and effective therapies,” noted Dr. Kieran Breen, Director of Research at Brain Tumor Research.

Check out the video above to learn more about both types of neurofibromatosis.

Sources: AAAS/Eurekalert! Via University of Plymouth, Oncogene

About the Author
BS
Experienced research scientist and technical expert with authorships on over 30 peer-reviewed publications, traveler to over 70 countries, published photographer and internationally-exhibited painter, volunteer trained in disaster-response, CPR and DV counseling.
You May Also Like
SEP 23, 2022
Neuroscience
'The Life of her Mind' Episode 1: Dr. Alice Dreger
SEP 23, 2022
'The Life of her Mind' Episode 1: Dr. Alice Dreger
The Life of Her Mind is a new video series focusing on the lives, perspectives, career pathways, and research of women w ...
SEP 25, 2022
Cell & Molecular Biology
New Imaging Tools Provide Insights Into Cell Stress
SEP 25, 2022
New Imaging Tools Provide Insights Into Cell Stress
In cells, active genes are transcribed into mRNA molecules, which can be edited and processed in a variety of ways, to u ...
SEP 28, 2022
Genetics & Genomics
Microprotein Mutations May Significantly Increase Alzheimer's Risk
SEP 28, 2022
Microprotein Mutations May Significantly Increase Alzheimer's Risk
The mitochondrion, commonly called the powerhouse of the cell, might be one of the best known organelles. This special o ...
OCT 21, 2022
Drug Discovery & Development
Gel-encased Radioactive Iodine Eliminates Pancreatic Cancer in Mice
OCT 21, 2022
Gel-encased Radioactive Iodine Eliminates Pancreatic Cancer in Mice
Researchers have developed a radioactive gel that eliminated pancreatic tumors in 80% of mice. The corresponding study w ...
OCT 27, 2022
Genetics & Genomics
Creating a Gene Therapy to Treat an Iron Storage Disorder
OCT 27, 2022
Creating a Gene Therapy to Treat an Iron Storage Disorder
Hereditary hemochromatosis (HH) is caused by genetic mutations that lead to the abnormal accumulation of iron in differe ...
NOV 14, 2022
Drug Discovery & Development
Experimental Opioid Vaccine Could Prevent Overdose Deaths
NOV 14, 2022
Experimental Opioid Vaccine Could Prevent Overdose Deaths
Researchers have developed a vaccine that may be able to block the opioid fentanyl from reaching the brain. They note th ...
Loading Comments...