Community Research and Development Information Service - CORDIS

FP5

PEMPHIGOIDS & IMMUNI Report Summary

Project ID: QLG1-CT-2001-02007
Funded under: FP5-LIFE QUALITY
Country: Switzerland

Characterization of the molecular interactions of the hemidesmosomal protein BP230 and BP180 with each other, with epidermal cytokeratins, and with laminin 5

Our studies have further our understanding of the molecular organization of hemidesmosomes and of their linkage with the cytoskeleton and the basement membrane. Hemidesmosomes are junctional adhesion complexes promoting epithelial-stromal cohesion in stratified epithelia, such as skin. Acquired and inherited (gene mutations) defects of components of hemidesmosomes and of their associated structures cause a number of life-threatening skin diseases, such as the autoimmune blistering diseases called pemphigoids and the inherited diseases known as epidermolysis bullosa. Our results have characterized the interactions of the hemidesmosomal components BP180 (BPAG2) and BP230 (BPAG1e) with either the cytokeratins K5/14 intermediate filament system or extracellular matrix protein laminin-5. The results indicate that sequences within the C-terminal domain of BP230 are critical for its association with K5/K14 keratins and that BP180 is able to directly bind to laminin-5. New knowledge about processes of basic biological importance, such as cell adhesion, maintenance of cytoarchitecture and cytoskeleton organization has been gained. This information contributes to a better understanding of the physiopathology of a group of acquired and inherited disorders of the skin characterized by skin blistering and fragility.
Clinically, it will be possible to better explain to patients affected by these rare diseases the mechanisms leading to skin blistering and fragility with failure of the skin to remains “sticked” together. Finally, it is expected that the acquired new knowledge will facilitate the development of better tailored therapies for a group of patients with acquired and congenital skin blistering disorders.

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Contact

Luca BORRADORI, (Chief of Unit)
Tel.: +41-22-3729427
Fax: +41-22-3729477
E-mail
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