New protein insight has potential to change patients lives

Researchers from the University of Plymouth and Plymouth Hospitals NHS Trust, supported by The Laura Crane Youth Cancer Trust and Brain Tumour Research have revealed the role of the cellular form of prion protein (PrPSc) in the development of Neurofibromatosis 2 (NF2) tumours with potential for other cancers.

NF2 is a hereditary condition that is characterised by the development of multiple tumours of the nervous systems such as schwannomas, meningiomas and ependymomas. Each tumour is associated with mutations in a gene coding for a tumour suppressor called Merlin.

This new research not only suggests potential in NF2, but also in other cancers with the same mutations; these include mesothelioma, breast cancer, colorectal carcinoma, melanoma, glioblastoma and spontaneous schwannomas and meningiomas occurring separately to NF2.

PrPC is ordinarily present in a healthy person’s nervous system, but absent in Creutzfeldt-Jakob prion disease patients who have ‘a pathological form of prion protein called scrapie prion protein (PrPSc).’ Most of the time PrPC is neuroprotective in adults, however, decidedly increased levels were found in several cancers such as glioblastoma, breast cancer, prostate and gastric cancer.

The scientists at the University of Plymouth and Plymouth Hospitals NHS Trust have developed a human cell culture model for schwannoma, comprised of human schwannoma cells that control healthy Schwann cells from donors. Using this prototype scientists were able to find - for the first time - that PrPC is overproduced in schwannoma because of Merlin deficiency. The researchers concluded that the overproduction of PrPC correlates with tumour growth and patient prognosis due to Merlin deficiency.

The significance of this new research lies with the ability to identify existing drugs that are able to manage protein overproduction. Already, researchers have managed to identify such drugs that are currently being used to treat other conditions not related to NF2. Thus, because these drugs have already been approved, there is scope for them to be fast-tracked into clinical studies for NF2.

Dr Sylwia Ammoun, Senior Research Fellow in Clinical Neurobiology at the Plymouth University Peninsula Schools of Medicine and Dentistry - internationally-recognised and run by Professor Oliver Hanemann - said of the research:

“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 tumours, adds yet more to the significance and excitement of our findings.”

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