79085 Freiburg, de
+49 (761) 270-67010
To Prevent Cancer in Butterfly Disease Patients
Epidermolysis Bullosa, also known as butterfly disease, is a genetic skin condition. The skin of patients with this disease is as fragile as the wings of a butterfly. It blisters easily in response to minor injury or friction such as rubbing or scratching. Furthermore, the patients develop chronic wounds that are not healing and their fingers and toes fuse, for example. The condition is caused by a mutation of the gene COL7A1, which contains the blueprint for the protein collagen VII. This protein helps to bind the epidermis and the dermis, two layers of the skin, together. In RDEB patient, collagen VII is completely absent and, therefore, the skin becomes fragile.
So far, little was known about the molecular mechanisms connecting squamous cell carcinomas and RDEB. The Freiburg research team discovered that the cancer progresses rapidly in RDEB patients, because the repeated mechanical injury alters the dermis. The amount of proteins called pro-fibrotic growth factors increases, thereby increasing stiffness of the dermis. This environment helps the tumour cells to spread.
The researchers have also identified mechanisms that could be potential drug targets. For example, molecules that inhibit the growth factor TGF reduce the stiffness and the spread of the RDEB cancer cells. “The new knowledge we have gained facilitates the design of prophylactic and therapeutic measures for delaying tumour progression and extending cancer-free periods in RDEB,” says Venugopal Rao Mittapalli, the first author of the study.
Leena Bruckner-Tuderman is Director of the Department of Dermatology of the Medical Center – University of Freiburg. Dimitra Kiritsi, Venugopal Rao Mittapalli und Alexander Nyström are researchers in Bruckner-Tuderman’s group. Winfried Römer is juniorprofessor for Synthetic Biology of Signalling Processes at the Cluster of Excellence BIOSS Centre for Biological Signalling Studies and the Institute of Biology II of the University of Freiburg. Josef Madl is postdoctoral scientist in Römer’s research group.
Mittapalli VR, Madl J, Löffek S, Kiritsi D, Kern JS, Römer W, Nyström A, Bruckner-Tuderman L. (2016). Injury-Driven Stiffening of the Dermis Expedites Skin Carcinoma Progression. In: Cancer Res. 76(4):940-51. doi: 10.1158/0008-5472.CAN-15-1348.
The use of information published here for personal information and editorial processing is generally free of charge. Please clarify any copyright issues with the stated publisher before further use. In the event of publication, please send a specimen copy to email@example.com.