ABCMdb

PMID: 7531240

Zar H, Saiman L, Quittell L, Prince A
Binding of Pseudomonas aeruginosa to respiratory epithelial cells from patients with various mutations in the cystic fibrosis transmembrane regulator.
J Pediatr. 1995 Feb;126(2):230-3., [PubMed]

Sentences

No. Mutations Sentence Comment

50 ABCC7 p.Gly551Asp ABCC7 p.Gly551Asp ABCC7 p.Trp1282* ABCC7 p.Trp1282* ABCC7 p.Trp1282* ABCC7 p.Gly542* (yr) Sex Genotype Mutation defect disease* Kulczycki score (%) 1 13 F AF508/AF508 Processing Mild 80 35 2 21 F AF508/2xF508 Processing Mild 83 33 3 13 M 2xF508/AF508 Processing Mild 87 26 4 15 M AF508/AF508 Processing Mild 87 22 5 15 F AF508/AF508 Processing Moderate 60 21 6 28 M AF508/AF508 Processing Moderate 50 17 7 15 F AF508/AF508 Processing Moderate 60 12 8 29 M AF508/3849 + 10 Processing/protein synthesis Moderate 55 7 9 43 F WI282X/W1282X Protein synthesis Moderate 60 3 10 13 F 2xF508/W1282X Processing/protein synthesis Mild 88 13 11 16 M Unknown Unknown Mild 82 6 12 22 F AF508/G551D Processing/regulation Mild 80 4 13 19 M AF508/G551D Processing/regulation Mild 80 18 14 12 M G542X/3849 + 10 Protein synthesis Mild 87 18 15 60 M W1282X/unknown Protein synthesis Moderate 75 5 *Severityofpulmonarydiseasewasmeasuredwiththe Shwachman-Kulczyckiscoringsystem. binding, there was no significant correlation between pulmonary status and the quantity of adherent bacteria. Login to comment