Epidermolysis bullosa Network (EB) International Kindler Syndrome Network (Kindlernet): "Rare Skin Diseases- a systemic approach"

Joint Symposium of FRIAS LIFENET and Dermatology

Epidermolysis bullosa Network (EB Network) International Kindler Syndrome Network (Kindlernet) on "Rare Skin Diseases - a systemic approach"

9.00 Welcome and opening (L. Bruckner-Tuderman)

09:10 - 09:30 Overview of the Epidermolysis bullosa and Kindler syndrome networks (L. Bruckner-Tuderman, Freiburg)

09:30 - 10:40 EB Network progress reports (each 7 minutes + 3 minutes discussion)

Data base and communication struture (project 2) (T. Ganslandt, Erlangen)

Center for diagnostics and management of EB (project 3) (H. Schumann, Freiburg)

Analysis of a mouse model for EBS (project 4) (J. Arin, Cologne)

Cellular and molecular interactions of laminin 332 at the derma-epidermal junction: analysis of changes in JEB (project 5) (M. Aumailley, Collogne)

Suprastructural elements of the dermal-epidermal junction zone (project 6) (D. Behrens, Münster)

Modulation of the chaperone machinery by co-chaperones and small molecules for the therapy of EBS (project 7) (S. Löffek, Bonn)

Molecular disease mechanisms and novel therapeutic strategy for DEB (project 9) (J. Kern, Freiburg)

10:40 - 11:10 Coffee break

11:10 - 11:35 A function of plakophilin 1 in the regulation of translation (M. Hatzfeld, Halle)

11:35 - 12:00 Plet-1, an adhesion modulating proteins expressed by differentiated outer root sheath keratinocytes (A. Sonnenberg, Amsterdam)

12:00 - 13:00 Lunch

13:00 - 13:50 Kindlernet progress reports (each 7 minutes + 3 minutes discussion)

Physiological functions of epithelial kindlins (partner 1) (C. Has, Freiburg)

Pathogenic mechanisms and genotype-phenotype correlations in KS (partner 2) (G. Zambruno, Rome)

Genome-wide screening and mutations of candidate genes (partner 3) (J. Fischer, Evry)

Mouse models for KS, comparison of human and murine phenotypes (partner 4) (R. Fässler, Martinsried)

Characterization of focal adhesion-basement membrane interactions: Development of tools (partner 5) (J. Kempf, Münster)

13:50 - 14:15 Familial tumoral calcinosis: from the delineation of a rare phenotype to the pathogenesis of ectopic calcification (E. Sprecher, Tel Aviv)

14:15 - 14:45 Coffee Break

14:45 - 15:10 The reticular fibre network of the lymph node - a specialized extracellular matrix structure (L. Sorokin, Münster)

15:10 - 15:35 Bone marrow transplantation therapy for epidermolysis bullosa mouse model (H. Shimizu, Sapporo)

15:35 - 16:00 General discussion

16:00 - 16:30 Visit of lab and core facilities