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DebRA International Completed Research Projects       

Cancer Pathogenesis in EB Patients:  Assessing the Contribution of Activated Stromal Cells

Name of Researchers: Prof. Seth L Schor
Prof Robin A J Eady
Dr John A McGrath
Places of Research: The Dental School, University of Dundee
Approved by DebRA
Medical & Scientific Advisory Panel:
28 February 2000
Budget approved by
DebRA central Committee:
25 March 2000
Date Commenced 1 May 2000 Duration 1 Year

 

SUMMARY OF RESEARCH BEING UNDERTAKEN

Epidermolysis Bullosa is a set of genetically inherited conditions affecting 1 in at least 17,000 of the population. A fault in a gene causes the skin to be extremely fragile. The layers of the skin do not adhere properly and painful widespread blisters occur very easily. Currently there is no effective treatment. However it is widely anticipated that gene therapy will eventually provide lasting and effective treatments for patients for patients suffering from EB.

Patients with Recessive Dystrophic forms of EB (RDEB) are particularly prone to develop skin cancer. Such malignancies commonly appear at sites of recurrent wound healing and scarring. Unfortunately, very little is known about the chemical mechanisms which are responsible for this distressing situation.

Prof Schor has previously identified a novel chemical factor, referred to as MSF, which appears to play an important and hitherto unrecognised role in the control of both wound healing and cancer development in healthy individuals. On the basis of available information, the group believes that MSF may also play an important role in these two disease processes in patients with RDEB. In order to test this possibility, this research will measure MSF production by skin cells in RDEB patients in normal looking skin, as well as areas of active wound healing, chronic ulceration, heavy scarring and skin cancers.

Comparative information will be obtained from healthy individuals in order to ascertain how MSF expression may be altered in RDEB patients. Related laboratory studies will be concerned with discovering means whereby doctors may be able to control (either switch on or switch off) MSF expression in a manner which will simultaneously promote healthy wound healing and prevent cancer development in RDEB patients. These novel means of therapy may involve the application of active wound dressings, as well as the introduction of genes into patient skin cells (ie gene therapy) which will either modify the expression MSF or its activity.

This project brings together two groups, one in Dundee and the other in London, with considerable expertise in the clinical care of RDEB patients and relevant areas of scientific investigation.


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