SUMMARY OF RESEARCH BEING UNDERTAKEN

It has been observed that degradation of part of the structure of the skin, known as the extracellular matrix, can lead to the detachment and migration of cells. The researchers have shown that the absence of a specific protein, laminin 332, activates this process and that an enzyme, protease uPA, is involved.

The project aims to investigate whether the increased uPA activity that is noticed in junctional EB keratinocytes plays a role is reducing cell adhesion, leading to the blistering that is typical of the condition. This is supported by the observation that a similar uPA activation in a transgenic mouse is associated with symptoms similar to junctional EB.