Current research funded
Developing therapies to control wound healing in EB. Prof.
Irwin McLean, University of Dundee.
Prof. McLeans group discovered the genetic defect that causes a condition known as
LOC syndrome- a specific form of junctional EB. In LOC syndrome patients have a chronic
defect in wound healing they continually produce a type of abnormal tissue, called
granulation tissue, in their skin, eyes, mouth, throat and other sites. Granulation tissue
is also a problem in other types of EB and in more common conditions, including rheumatoid
arthritis, leg ulcers and so on. There is no effective drug at the present time
specifically to treat granulation tissue in any of these conditions.
Most LOC patients are lacking a small piece of protein found in the skin known as laminin
alpha-3a. Complete loss of laminin alpha-3a causes the severe Herlitz type of junctional
EB and loss of this small part of the protein causes LOC. Recently, the group identified
one LOC patient with a different type of mutation also affecting this same small part of
the laminin alpha-3a protein. This is strong evidence that this region of the laminin
alpha-3a protein, which is called the N domain, is a key signal that turns off granulation
tissue production at the end of the wound healing process. In LOC patients this signal is
absent and so granulation tissue continues to be made continuously. This discovery is very
exciting because it tells us for the first time how granulation tissue production is
turned off in the skin after a wound is healed and it also opens the way to develop drugs
to accelerate this process. This project builds on the discovery towards identifying such
drugs.
A novel treatment for wound healing in RDEB. Dr Abhay Pandit,
National University of Ireland, Galway. Funded by DEBRA Ireland.
The specific objective of this project is to treat patients with debilitating wounds by
bridging the gap between basic science on wound healing and the potential clinical
application for new technologies.
The central idea of this work is on engineering smart templates that guide the
regeneration of tissue. This smart template will be capable of sensing the
environment that it is in and taking necessary actions. The rationale of the project is
based on an understanding of the bodys natural processes and incorporates the use of
natural biodegradable materials and the signals that the human body deciphers. Hence, the
goal of this work is to provide, as part of a skin substitute, a dermal precursor that is
capable of directing true skin regeneration.
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