FFC#11/2015

Genetically diverse mice as innovative model for cystic fibrosis

FFC#11/2015

Genetically diverse mice as innovative model for cystic fibrosis

PRINCIPAL INVESTIGATOR

Nicola Ivan Lorè (Divisione di Immunologia, Trapianti e Malattie Infettive – Istituto Scientifico San Raffaele, Milano)

RESEARCHERS

5

CATEGORY

AREA 3 Bronchopulmonary infection

DURATION

2 years

GOAL

€ 65.000 €

RESULTS

Selection of candidate resistant (R) and susceptible (S) CC lines to P. aeruginosa infection was performed using a data sets, including survival rate, bacterial load and inflammatory response. By backcross breeding, the CFTR-F508del mutation have been introduced in two (R and S) CC backgrounds. Phenotypic characterization of CC lines showed that the host genetic diversity influences the progress and severity of P. aeruginosa airway infection. Based on these results, F508del of C57Bl/6 mice has been backcrossed into two CC lines with deviant phenotypes, resistant CC-R and susceptible CC-S mouse strains for airway disease. Two new CF mouse models were generated and will be used to study the role of the genetic background in the CF pathogenesis. Through the extension project (FFC#4/2017), researchers plan to analyze pathological mechanisms induced spontaneously or after infection by P. aeruginosa; inflammatory and microbiological profile will be studied and histopathology will be associated with quantitative analyses to highlight cellular and structural modifications in the lung.

OTHER RESULTS

FFC #3/2024

Two molecules are effective in activating Heat Shock Proteins and enhancing the action of CFTR correctors with the F508del mutation in vitro.

FFC#5/2024

Some peptide nucleic acids (PNAs) re-sensitise Pseudomonas aeruginosa to the antibiotic meropenem in vitro and reduce its virulence.

FFC#1/2023

Tezacaftor, one of the components of Kaftrio, induces an accumulation of dihydroceramides both in vitro and in vivo in animal models