FFC#10/2016

Modulation of proteinkinase CK2 in the regulation of chaperone machinery leading the F508del-CFTR fate

FFC#10/2016

Modulation of proteinkinase CK2 in the regulation of chaperone machinery leading the F508del-CFTR fate

PRINCIPAL INVESTIGATOR

Mauro Salvi (Dipartimento di Scienze Biomediche, Università di Padova)

RESEARCHERS

4

CATEGORY

AREA 1 Therapies to correct the underlying defect

DURATION

1 year

GOAL

€ 30.000 €

RESULTS

The researchers performed in vitro and in cells studies to demonstrate how CK2 can affect F508del stability using transfections, specific CK2 kinase inhibitors and CK2 knockout cells.
They have demonstrated that CK2 inhibition may represent an important tool to reduce the expression of HSP27, a chaperone that specifically recognizes F508del leading to its ubiquitylation and degradation. Moreover, the detailed mechanisms by which HSP27 expression is regulated by CK2 has been elucidated. However, the direct correlation between CK2-HSP27-CFTR-F508del is still lacking and is under study. The extension project FFC#12/2017 aims at providing the rationale for the use of CK2 inhibitors in combinatory therapies with correctors or proteostasis regulators for the rescue and stabilization of F508delCFTR at the plasma membrane.

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