Please use this identifier to cite or link to this item: https://hdl.handle.net/2440/7008
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Type: Journal article
Title: Sulfation of chondroitin/dermatan sulfate by cystic fibrosis pancreatic duct cells is not different from control cells
Author: Hill, W.
Harper, G.
Rozaklis, T.
Hopwood, J.
Citation: Biochemical and molecular medicine, 1997; 62(1):85-94
Publisher: ACADEMIC PRESS INC ELSEVIER SCIENCE
Issue Date: 1997
ISSN: 1077-3150
1095-5577
Statement of
Responsibility: 
Warren G. Hill, Gregory S. Harper, Tina Rozaklis, John J. Hopwood
Abstract: Cystic fibrosis is associated with mutations of the cystic fibrosis transmembrane conductance regulator (CFTR), a cAMP-regulated plasma membrane chloride channel. Cystic fibrosis patients have been reported to possess elevated sulfation of glycoconjugates, which may contribute to the pathogenesis of the disease. Sulfation of glycosaminoglycans by a cystic fibrosis pancreatic adenocarcinoma cell line homozygous for DeltaF508 (CFPAC-1), a control pancreatic cell line (PANC-1), two CFPAC-1 cell lines transfected with the gene for CFTR (PLJ-CFTR-4.7, TR20), and a mock-transfected CFPAC-1 control (PLJ-6) was investigated. Cells were radiolabeled with [35S]sulfate and [3H]glucosamine, and glycosaminoglycans secreted into the medium after 24 and 72 h were isolated. Chondroitinase ABC digestion of chondroitin/dermatan sulfate allowed the recovery of disaccharides which were analyzed for their degree of sulfation by strong anion-exchange HPLC. No differences in the extent of sulfation by any of the cell lines were noted. However, glycoaminoglycans synthesized by cystic fibrosis cells consistently exhibited twofold higher [35S]-sulfate:[3H]glucosamine ratios than the controls. We conclude that CFTR plays no role in the sulfation of chondroitin/dermatan sulfate by pancreatic cells and that isotope incorporation ratios alone are insufficient evidence of changes in sulfation levels.
Keywords: cystic fibrosis
CFTR
sulfation
glycosaminoglycans
pancreatic cells
Rights: Copyright © 1997, Elsevier
DOI: 10.1006/bmme.1997.2625
Appears in Collections:Aurora harvest
Paediatrics publications

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