ABCMdb

PMID: 16272177

Tabary O, Corvol H, Boncoeur E, Chadelat K, Fitting C, Cavaillon JM, Clement A, Jacquot J
Adherence of airway neutrophils and inflammatory response are increased in CF airway epithelial cell-neutrophil interactions.
Am J Physiol Lung Cell Mol Physiol. 2006 Mar;290(3):L588-96. Epub 2005 Nov 4., [PubMed]

Sentences

No. Mutations Sentence Comment

66 ABCC7 p.Gly551Asp ABCC7 p.Asn1303Lys ABCC7 p.Gly542* ABCC7 p.Arg1066Cys ABCC7 p.Arg560Cys Sex Age, yr CF Genotype Clinical Score FVC, % FEV1, % Pseudomonas aeruginosa Colonization 1 M 15 ⌬F508/⌬F508 30 32 21 yes 2 M 16 ⌬F508/⌬F508 60 70 43 yes 3 M 17 ⌬F508/⌬F508 30 41 23 yes 4 F 4 ⌬F508/⌬F508 80 NA NA no 5 F 16 ⌬F508/⌬F508 50 53 41 yes 6 M 15 ⌬F508/⌬F508 30 33 20 yes 7 M 13 ⌬F508/⌬F508 50 61 37 yes 8 M 15 ⌬F508/⌬F508 70 70 50 no 9 F 21 R1066C/NI 50 60 77 yes 10 F 14 2183A/2183A 50 48 39 yes 11 M 14 G542X/2176insC 30 34 25 yes 12 M 17 ⌬F508/R560C 30 47 27 yes 13 M 17 IVS8 (5T)/IVS8 (5T) NA NA NA yes 14 F 18 3906insT/1609⌬CA 40 47 30 yes 15 F 13 G551D/S1235Rϩ5T 70 84 86 no 16 F 15 N1303K/347 del70 70 44 35 yes Clinical score, Schwachman-Kulczycki score; FVC, forced vital capacity; FEV1, forced expiratory volume in 1 s; M, male; F, female; NA, not applicable. Login to comment 79 ABCC7 p.Trp1282* Three different types of transformed human bronchial epithelial cells were used in the present study: IB3-1 cells that express mutant CFTR (genotype ⌬F508/W1282X); C38 cells, the "corrected" CFTR-complemented cell line with a functional CFTR that is derived from IB3-1 cells and stably transfected with an episomal, truncated of CFTR; and BEAS-2B cells that express wild-type CFTR (46, 71). Login to comment