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PMID: 12386452
Rhim AD, Stoykova L, Glick MC, Scanlin TF
Terminal glycosylation in cystic fibrosis (CF): a review emphasizing the airway epithelial cell.
Glycoconj J. 2001 Sep;18(9):649-59.,
[PubMed]
Sentences
No.
Mutations
Sentence
Comment
97
ABCC7 p.Trp1282*
X
ABCC7 p.Trp1282* 12386452:97:759
status:
NEW
view ABCC7 p.Trp1282* details
Modulation of CF terminal glycosylation phenotype by CFTR expression Cell type1 Transfection2 Compared with non-transfected cells3 Reference CF/T1 wtCFTR ↓ Fucα1,3/4GlcNAc; ↑ SA [62] wtCFTR ↓ Fucα1,3/4GlcNAc; ↑ SA [4] wtCFTR ↓ Fucα1,3/4GlcNAc; ↑ Fucα1,2Gal [17] wtCFTR ↓ PNA4 [29] IB3 wtCFTR ↓ PNA [28] wtCFTR ↓ PNA [29] wtCFTR ↓ asialo GM1 [27] CFPAC wtCFTR ↓ asialo GM1 [27] 9/HTEo- CFTR R-domain ↑ asialo GM1 [63] F508 ↓ SNA5 [26] CFTR R-domain ↓ SNA [26] C127 F508 ↓ WGA6 [25] 1 Cell line in which the experiments were conducted: CF/T1, immortalized CF tracheal epithelial cells ( F508/ F508); IB3, CF bronchial cells ( F508/
W1282X
); CFPAC, immortalized CF pancreatic epithelial cells; 9/HTEo-, immortalized non-CF human tracheal epithelial cells with constitutive expression of wtCFTR; C127, mouse mammary epithelial cells.
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