Glycosylation and the cystic fibrosis transmembrane conductance regulator

Respiratory Research

Mary Catherine Glick¹  Thomas F Scanlin¹ 

[1]Cystic Fibrosis Center and Department of Pediatrics, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Abramson Pediatric Research Center, Philadelphia, Pennsylvania, USA

关键词: polylactosamine;    oligosaccharides;    ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR);   

Others  :  1227433
DOI  :  10.1186/rr69

 received in 2001-05-30, accepted in 2001-06-27,  发布年份 2001

【 摘 要 】

The cystic fibrosis transmembrane conductance regulator (CFTR) has been known for the past 11 years to be a membrane glycoprotein with chloride channel activity. Only recently has the glycosylation of CFTR been examined in detail, by O'Riordan et al in Glycobiology. Using cells that overexpress wild-type (wt)CFTR, the presence of polylactosamine was noted on the fully glycosylated form of CFTR. In the present commentary the results of that work are discussed in relation to the glycosylation phenotype of cystic fibrosis (CF), and the cellular localization and processing of ΔF508 CFTR. The significance of the glycosylation will be known when endogenous CFTR from primary human tissue is examined.

【 授权许可】

   
2001 BioMed Central Ltd

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