THE LONG PENTRAXIN PTX3 IN HOST RESISTANCE TO INFECTION AND AS A CANDIDATE DIAGNOSTIC AND THERAPEUTIC AGENT IN CYSTIC FIBROSIS
- 3 Years 2006/2009
- 394.999€ Total Award
The prototypic long pentraxin PTX3, discovered by the applicant group, is a non-redundant component of innate immunity against selected pathogens, including Pseudomonas aeruginosa, a bacterium which plays a key role in the pathology of patients with cystic fibrosis. Studies conducted by the applicant have defined the role of this molecule in immunological mechanisms and have generated unique tools to investigate its function. The purpose of this project will be to translate the knowledge on PTX3 in the clinical context of genetic diseases. Preclinical studies will be conducted to define the potential of PTX3 as a prophylactic/therapeutic agent against P. aeruginosa, a formidable therapeutic challenge in cystic fibrosis. Recent results indicate that PTX3 can represent a new marker, independent of classic molecules such as C reactive protein, in infectious and inflammatory pathology. On this basis, PTX3 levels will be investigated in patients with cystic fibrosis with or without P. aeruginosa infection, in an effort to define the utility of this molecule in the diagnosis and monitoring of these subjects. These studies, together with the preclinical investigations, could provide a basis to assess PTX3 administration as a prophylactic and/or therapeutic agent against P. aeruginosa. Moreover, the non-redundant role played by PTX3 in innate immunity against selected pathogens raises the possibility that specific PTX3 deficiency may account for increased susceptibility to P. aeruginosa infection in patients with cystic fibrosis. The search and identification of genetic defects in PTX3 production will provide a basis for substitution therapy.