Altered terminal glycosylation and the pathophysiology of CF lung disease

Andrew D. Rhim; Lidia I. Stoykova; Arvind J. Trindade; Mary Catherine Glick; Thomas F. Scanlin

doi:10.1016/j.jcf.2004.05.021

Altered terminal glycosylation and the pathophysiology of CF lung disease

Andrew D. Rhim, Lidia I. Stoykova, Arvind J. Trindade, Mary Catherine Glick, Thomas F. Scanlin

Research output: Contribution to journal › Article › peer-review

21 Scopus citations

Abstract

Altered terminal glycosylation, with increased fucosylation and decreased sialylation, is a hallmark of the cystic fibrosis (CF) glycosylation phenotype. The glycosylation phenotype of CF airway epithelial cells has been modulated by the expression of wtCFTR. Understanding the effects of mutant CFTR on glycosylation may provide further insight into the regulation of glycoconjugate processing as well as new approaches to the therapy of CF.

Original language	English (US)
Pages (from-to)	95-96
Number of pages	2
Journal	Journal of Cystic Fibrosis
Volume	3
Issue number	SUPPL. 2
DOIs	https://doi.org/10.1016/j.jcf.2004.05.021
State	Published - Aug 2004
Externally published	Yes

All Science Journal Classification (ASJC) codes

Pediatrics, Perinatology, and Child Health
Pulmonary and Respiratory Medicine

Keywords

CFTR
Cystic fibrosis
Fucosylation
Golgi
Terminal glycosylation

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10.1016/j.jcf.2004.05.021

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title = "Altered terminal glycosylation and the pathophysiology of CF lung disease",

abstract = "Altered terminal glycosylation, with increased fucosylation and decreased sialylation, is a hallmark of the cystic fibrosis (CF) glycosylation phenotype. The glycosylation phenotype of CF airway epithelial cells has been modulated by the expression of wtCFTR. Understanding the effects of mutant CFTR on glycosylation may provide further insight into the regulation of glycoconjugate processing as well as new approaches to the therapy of CF.",

keywords = "CFTR, Cystic fibrosis, Fucosylation, Golgi, Terminal glycosylation",

author = "Rhim, {Andrew D.} and Stoykova, {Lidia I.} and Trindade, {Arvind J.} and Glick, {Mary Catherine} and Scanlin, {Thomas F.}",

note = "Funding Information: Supported by the NIH (DK-07748 to A.D.R.) and the Cystic Fibrosis Foundation (SCANLI00Z0 to T.F.S.).",

year = "2004",

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doi = "10.1016/j.jcf.2004.05.021",

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journal = "Journal of Cystic Fibrosis",

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T1 - Altered terminal glycosylation and the pathophysiology of CF lung disease

AU - Rhim, Andrew D.

AU - Stoykova, Lidia I.

AU - Trindade, Arvind J.

AU - Glick, Mary Catherine

AU - Scanlin, Thomas F.

N1 - Funding Information: Supported by the NIH (DK-07748 to A.D.R.) and the Cystic Fibrosis Foundation (SCANLI00Z0 to T.F.S.).

PY - 2004/8

Y1 - 2004/8

N2 - Altered terminal glycosylation, with increased fucosylation and decreased sialylation, is a hallmark of the cystic fibrosis (CF) glycosylation phenotype. The glycosylation phenotype of CF airway epithelial cells has been modulated by the expression of wtCFTR. Understanding the effects of mutant CFTR on glycosylation may provide further insight into the regulation of glycoconjugate processing as well as new approaches to the therapy of CF.

AB - Altered terminal glycosylation, with increased fucosylation and decreased sialylation, is a hallmark of the cystic fibrosis (CF) glycosylation phenotype. The glycosylation phenotype of CF airway epithelial cells has been modulated by the expression of wtCFTR. Understanding the effects of mutant CFTR on glycosylation may provide further insight into the regulation of glycoconjugate processing as well as new approaches to the therapy of CF.

KW - CFTR

KW - Cystic fibrosis

KW - Fucosylation

KW - Golgi

KW - Terminal glycosylation

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JO - Journal of Cystic Fibrosis

JF - Journal of Cystic Fibrosis

IS - SUPPL. 2

ER -

Altered terminal glycosylation and the pathophysiology of CF lung disease

Abstract

All Science Journal Classification (ASJC) codes

Keywords

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