Cellosaurus cell line IB3-1 (CVCL_0338)

• Mutation; HGNC; 1884; CFTR; Simple; p.Phe508del (c.1521_1523delCTT); ClinVar=VCV000007105; Zygosity=Heterozygous (PubMed=15463957).
  
• Mutation; HGNC; 1884; CFTR; Simple; p.Trp1282Ter (c.3846G>A); ClinVar=VCV000007129; Zygosity=Heterozygous (PubMed=15463957).
  

PubMed=1849726; DOI=10.1165/ajrcmb/4.4.313
Zeitlin P.L., Lu L., Rhim J.S., Cutting G.R., Stetten G., Kieffer K.A., Craig R., Guggino W.B.
A cystic fibrosis bronchial epithelial cell line: immortalization by adeno-12-SV40 infection.
Am. J. Respir. Cell Mol. Biol. 4:313-319(1991)

PubMed=1380129; DOI=10.1038/358581a0
Egan M., Flotte T.R., Afione S.A., Solow R., Zeitlin P.L., Carter B.J., Guggino W.B.
Defective regulation of outwardly rectifying Cl- channels by protein kinase A corrected by insertion of CFTR.
Nature 358:581-584(1992)

PubMed=7679117; DOI=10.1016/S0021-9258(18)53762-5
Flotte T.R., Afione S.A., Solow R., Drumm M.L., Markakis D., Guggino W.B., Zeitlin P.L., Carter B.J.
Expression of the cystic fibrosis transmembrane conductance regulator from a novel adeno-associated virus promoter.
J. Biol. Chem. 268:3781-3790(1993)

PubMed=15463957; DOI=10.1016/j.jcf.2004.05.040
Gruenert D.C., Willems M., Cassiman J.-J., Frizzell R.A.
Established cell lines used in cystic fibrosis research.
J. Cyst. Fibros. 3:191-196(2004)

PubMed=16512671; DOI=10.1021/pr050319o
Singh O.V., Vij N., Mogayzel P.J. Jr., Jozwik C., Pollard H.B., Zeitlin P.L.
Pharmacoproteomics of 4-phenylbutyrate-treated IB3-1 cystic fibrosis bronchial epithelial cells.
J. Proteome Res. 5:562-571(2006)

PubMed=18441018; DOI=10.1194/jlr.M700388-JLR200
Andersson C., Al-Turkmani M.R., Savaille J.E., Alturkmani R., Katrangi W., Cluette-Brown J.E., Zaman M.M., Laposata M., Freedman S.D.
Cell culture models demonstrate that CFTR dysfunction leads to defective fatty acid composition and metabolism.
J. Lipid Res. 49:1692-1700(2008)