PW01-035 – Mutations in PB30.2D and complexing with caspase-1

Introduction Mutations M680I, M694V and V726A of Pyrin the product of MEFV gene are localized at the domain B30.2 (PB30.2D) and responsible for manifestation of the most widespread and severe forms of FMF. From the other hand, it is well known that malfunction of the pyrin-caspase-1complex is the main reason for inflammation during FMF. Therefore, we suggest that comparative investigation of normal and mutated pyrin and caspase-1 interaction will help to reveal possible link between those mutations and structural changes that influence formation of pyrincaspase-1 complex.