FFC#01/2015

Relationship between mitochondria and F508del-CFTR in Cystic Fibrosis

FFC#01/2015

Relationship between mitochondria and F508del-CFTR in Cystic Fibrosis

PRINCIPAL INVESTIGATOR

Anna Atlante (IBBE – Istituto delle Biomembrane e Bioenergetica, CNR Bari)

RESEARCHERS

13

CATEGORY

AREA 1 Therapies to correct the underlying defect

DURATION

2 years

GOAL

€ 25.000 €

RESULTS

The project inquired bacteria population of high and lower respiratory tract and gave evidence that in patients Pa chronic infection colonization in the lung seems to prevent colonization by other bacterial species; in patients without chronic Pa infection, colonization was seen by different emerging pathogens for which there is still no clear pathogenic role. In pediatric patients, St. aureus appeared the predominant bacterial specie. Interestingly a high percentage of patients were colonized in the nasal and pulmonary sites by the same genetically related bacterial species, indicating that it is the same clone and confirms their passage from the high to the lower airways. About 40% of toothbrushes were colonized by bacteria and carry them in the oral cavity and thus potentially in the lower respiratory tract. Guidelines for collection and monitoring of the bacterial flora of the nasal/ paranasal reservoir of selected patients FC are proposed, moreover protocols for the eradication of upper respiratory tract bacteria in order to prevent their passage to the lower respiratory tract; and finally treatment and conservation protocols of toothbrushes in order to avoid contact with environmental bacteria.


Congress abstracts

– Sandri A, Cazzarolli C, Burlacchini G et al. “Human reservoirs of pathogens colonising the airways of cystic fibrosis patients” 41st European Cystic Fibrosis Conference, Belgrade, Serbia, 6-9 June 2018

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