FFC#10/2018

Dissecting the mechanism of action of the TG2 inhibitor cysteamine on Cystic Fibrosis

FFC#10/2018

Dissecting the mechanism of action of the TG2 inhibitor cysteamine on Cystic Fibrosis

PRINCIPAL INVESTIGATOR

Mauro Piacentini (Università Roma Tor Vergata, Dip. Biologia)

Partner

Luigi Maiuri (Istituto Europeo Ricerca Fibrosi Cistica – IERFC c/o Istituto San Raffaele, Milano); Giovanni Delogu (Università Cattolica del Sacro Cuore, Fondazione Policlinico Gemelli, Istituto di Microbiologia, Roma)

RESEARCHERS

16

CATEGORY

AREA 1 Therapies to correct the underlying defect

DURATION

1 year

GOAL

€ 40.000 €

RESULTS

The findings oh this study indicate that cysteamine can improve the clearance of pathogenic mycobacteria such as Mycobacterium abscessus and Pseudomonas aeruginosa. Moreover, it was found that TG2 is able to control the innate immune response by regulating type 1 interferon production, thus possibly explaining the negative role of the enzyme in the infection process. These results will define the molecular basis that supports the use of cysteamine not only as a CFTR corrector but also as a promising therapy against bacterial opportunistic infections. To understand the molecular pathway involved in bacterial infection could provide new possible targets and the possibility to define novel strategies aimed to improve the health care of CF patients.

OTHER RESULTS

FFC#1/2023

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

FFC#4/2023

Pseudopaline–aztreonam conjugates exhibited enhanced antimicrobial activity against Pseudomonas aeruginosa compared to aztreonam alone

FFC#10/2023

Several drugs already approved for human use inhibit the growth of P. aeruginosa, its virulence, or its ability to form biofilms in vitro