ABCMdb

PMID: 11809765

Poschet JF, Skidmore J, Boucher JC, Firoved AM, Van Dyke RW, Deretic V
Hyperacidification of cellubrevin endocytic compartments and defective endosomal recycling in cystic fibrosis respiratory epithelial cells.
J Biol Chem. 2002 Apr 19;277(16):13959-65. Epub 2002 Jan 24., 2002-04-19 [PubMed]

Sentences

No. Mutations Sentence Comment

20 ABCC7 p.Trp1282* IB3-1 is a human bronchial epithelial cell line derived from a CF patient with a ⌬F508/W1282X CFTR mutant genotype (21). Login to comment 96 ABCC7 p.Trp1282* The endosomal (cellubrevin) and plasma membrane (GPI)-targeted pHluorin GFP probes were transfected into well characterized human bronchial epithelial cells (21, 22): IB3-1 (from a compound heterozygote CFTR ⌬F508/W1282X CF patient), C38 (IB3-1 cells corrected with a functional CFTR lacking the first ecto-loop), and S9 (IB3-1 cells corrected with a full size functional CFTR cDNA). Login to comment 194 ABCC7 p.Trp1282* TABLE I Hyperacidification of TGN38 and cellubrevin-labeled compartments in CF respiratory epithelial cells Cell linea Apparent pHb mean Ϯ S.E. IB3-1 (mutant) pH 6.2 Ϯ 0.1 (n ϭ 19) C38 (corrected) pH 6.7 Ϯ 0.1 (n ϭ 15) S9 (corrected) pH 6.7 Ϯ 0.1 (n ϭ 32) CFT1 (mutant) pH 6.1 Ϯ 0.1 (n ϭ 15) CFT1-LCFSN (corrected) pH 6.6 Ϯ 0.03 (n ϭ 14) CFT1-LC3 (mutant) pH 6.0 Ϯ 0.1 (n ϭ 15) CFT1-⌬F508 (mutant) pH 6.2 Ϯ 0.1 (n ϭ 19) a IB3-1 is a human bronchial cell line derived from a CF patient with a ⌬F508/W1282X CFTR mutant genotype (21). Login to comment