ABCMdb

PMID: 23617438

Van de Weert-van Leeuwen PB, Van Meegen MA, Speirs JJ, Pals DJ, Rooijakkers SH, Van der Ent CK, Terheggen-Lagro SW, Arets HG, Beekman JM
Optimal complement-mediated phagocytosis of Pseudomonas aeruginosa by monocytes is cystic fibrosis transmembrane conductance regulator-dependent.
Am J Respir Cell Mol Biol. 2013 Sep;49(3):463-70. doi: 10.1165/rcmb.2012-0502OC., [PubMed]

Sentences

No. Mutations Sentence Comment

54 ABCC7 p.Gly542* ABCC7 p.Gly542* Immune cells were isolated from healthy control subjects and patients with CF who carried a G542X/G54X, G542X/F508 del mutation or F508 del/F508 del mutation. Login to comment 64 ABCC7 p.Gly542* ABCC7 p.Gly542* To confirm CFTR antibody specificity, whole-cell CFTR expression levels were compared with those of isotype control antibodies, and CF cells were derived from a subject with two CFTR nonsense mutations (G542X/G542X), a mutation of which it is known that these cells have no expression of CFTR. Login to comment 86 ABCC7 p.Gly542* ABCC7 p.Gly542* To confirm CFTR specificity further, whole-blood immune cells were isolated from a healthy control subject and a CF patient with two nonsense mutations (G542X/ G542X), with an expectation of absent expression of CFTR. Login to comment 88 ABCC7 p.Gly542* ABCC7 p.Gly542* CFTR expression in nasal epithelial cells from a healthy control subject, but not in a patient with CF (G542X/G542X), further supported the contention that our staining procedure could detect CFTR (Figure 1C). Login to comment 93 ABCC7 p.Gly542* ABCC7 p.Gly542* We confirmed staining specificity in CD141 monocytes from a healthy control subject and a patient with CF and a homozygous G542X/G542X mutation (Figure 2B). Login to comment 118 ABCC7 p.Gly542* ABCC7 p.Gly542* (B) Whole-cell CFTR (red) expression in peripheral blood immune cells of a healthy control subject, compared with a patient with a G542X/G542X mutation, using L12B4 and 570. Login to comment 119 ABCC7 p.Gly542* ABCC7 p.Gly542* (C) Whole-cell CFTR (green) expression in nasal epithelial cells of a healthy control subject and a patient with a G542X/G542X mutation, using L12B4. Login to comment 128 ABCC7 p.Gly542* (B) Flow cytometric analysis of whole-cell CFTR expression in monocytes of a healthy control subject compared with a patient with CF and carrying a homozygous G542X mutation, using the CFTR mAbs 570 and L12B4. Login to comment