Publications
The following publications highlight some of the work of the CFIT Program. Click the title of the article to link to the Pubmed entry or click the link at the end of the entry, if available, for direct access to the full article.
The CF Canada-SickKids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF.
Eckford PDW, McCormack J, Munsie L, He G, Stanojevic S, Pereira SL, Ho K, Avolio J, Bartlett C, Yang JY, Wong AP, Wellhauser L, Huan LJ, Jiang JX, Ouyang H, Du K, Klingel M, Kyriakopoulou L, Gonska T, Moraes TJ, Strug LJ, Rossant J, Ratjen F, Bear CE.
J Cyst Fibros. 2018 Apr 20. pii: S1569-1993(18)30086-9. doi: 10.1016/j.jcf.2018.03.013. Open access to article here.
PMID: 29685812
Functional rescue of c.3846G>A (W1282X) in patient-derived nasal cultures achieved by inhibition of nonsense mediated decay and protein modulators with complementary mechanisms of action.
Laselva O, Eckford PDW, Bartlett C, Ouyang H, Gunawardena TN, Gonska T, Moraes TJ, Bear CE.
J Cyst Fibros. 2019 Dec 9. pii: S1569-1993(19)30980-4. doi: 10.1016/j.jcf.2019.12.001. [Epub ahead of print] Open access to article here.
PMID: 31831337
Genetic association and transcriptome integration identify contributing genes and tissues at cystic fibrosis modifier loci.
Gong J, Wang F, Xiao B, Panjwani N, Lin F, Keenan K, Avolio J, Esmaeili M, Zhang L, He G, Soave D, Mastromatteo S, Baskurt Z, Kim S, O’Neal WK, Polineni D, Blackman SM, Corvol H, Cutting GR, Drumm M, Knowles MR, Rommens JM, Sun L, Strug LJ.
PLoS Genet. 2019 Feb 26;15(2):e1008007. doi: 10.1371/journal.pgen.1008007. eCollection 2019 Feb. Open access to article here.
PMID: 30807572
ORKAMBI-Mediated Rescue of Mucociliary Clearance in Cystic Fibrosis Primary Respiratory Cultures Is Enhanced by Arginine Uptake, Arginase Inhibition, and Promotion of Nitric Oxide Signaling to the Cystic Fibrosis Transmembrane Conductance Regulator Channel.
Wu YS, Jiang J, Ahmadi S, Lew A, Laselva O, Xia S, Bartlett C, Ip W, Wellhauser L, Ouyang H, Gonska T, Moraes TJ, Bear CE.
Mol Pharmacol. 2019 Oct;96(4):515-525. doi: 10.1124/mol.119.117143. Epub 2019 Aug 19.
PMID: 31427400