Cystic fibrosis (CF), one of the most common lethal hereditary diseases of white European populations, is caused by loss-of-function mutations in the CF Transmembrane conductance Regulator (CFTR) gene. One of the main causes of mortality is the onset of CF lung disease, which is characterized by chronic infection and inflammation resulting in the progressive remodelling, irreversible damage and fibrosis of the airways. An increasing number of studies indicate that sphingolipids are crucial players in pulmonary manifestations of CF, even if their direct involvement in CF lung disease is still unclear. In this review, we give an overview of the role of sphingolipids in CF pulmonary disease, focusing on the relationship between glycosphingolipids and lung inflammation, which represents the main hallmark of this disease.

Unravelling the role of sphingolipids in cystic fibrosis lung disease / M. Aureli, D. Schiumarini, N. Loberto, R. Bassi, A. Tamanini, G. Mancini, M. Tironi, S. Munari, G. Cabrini, M.C. Dechecchi, S. Sonnino. - In: CHEMISTRY AND PHYSICS OF LIPIDS. - ISSN 0009-3084. - 200(2016 Aug), pp. 94-103. [10.1016/j.chemphyslip.2016.08.002]

Unravelling the role of sphingolipids in cystic fibrosis lung disease

M. Aureli
Primo
;
D. Schiumarini
Secondo
;
N. Loberto;R. Bassi;G. Mancini;S. Sonnino
Ultimo
2016

Abstract

Cystic fibrosis (CF), one of the most common lethal hereditary diseases of white European populations, is caused by loss-of-function mutations in the CF Transmembrane conductance Regulator (CFTR) gene. One of the main causes of mortality is the onset of CF lung disease, which is characterized by chronic infection and inflammation resulting in the progressive remodelling, irreversible damage and fibrosis of the airways. An increasing number of studies indicate that sphingolipids are crucial players in pulmonary manifestations of CF, even if their direct involvement in CF lung disease is still unclear. In this review, we give an overview of the role of sphingolipids in CF pulmonary disease, focusing on the relationship between glycosphingolipids and lung inflammation, which represents the main hallmark of this disease.
Cystic fibrosis; Inflammation; Lipid rafts; Sphingolipids
Settore BIO/10 - Biochimica
ago-2016
Article (author)
File in questo prodotto:
File Dimensione Formato  
schhiumarini1-s2.0-S0009308416301104-main.pdf

accesso riservato

Tipologia: Post-print, accepted manuscript ecc. (versione accettata dall'editore)
Dimensione 1.62 MB
Formato Adobe PDF
1.62 MB Adobe PDF   Visualizza/Apri   Richiedi una copia
Pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/434743
Citazioni
  • ???jsp.display-item.citation.pmc??? 15
  • Scopus 25
  • ???jsp.display-item.citation.isi??? 25
social impact