Mucus Glycoproteins Secreted by Respiratory Epithelial Tissue from Cystic Fibrosis Patients

Frates, Ralph C.; Kaizu, Tokio

doi:10.1203/00006450-198301000-00006

Cited by 76 publications

(35 citation statements)

References 11 publications

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“…Such studies have identified CF-associated biochemical alterations in secreted glycoproteins, including increased sulfation and fucosylation together with decreased sialylation. [30][31][32][33][34][35][36][37] However, the potential for secondary effects of bacterial infection in these studies has caused considerable debate in this area, 38 as indicated by a report which shows no abnormalities in the biochemical properties of CF mucus. 39 Recently, the use of a human bronchial xenograft model of CF and non-CF airways has allowed for the comparison of mucin biochemical properties in the absence of bacterial infection and goblet cell hyperplasia.…”

Section: Heterogeneity Of Cftr-expressing Cell Types In the Lungmentioning

confidence: 99%

“…57 This mechanism attempts to explain that the increased sulfation of glycoproteins arises from an increase in the concentration of intravesicular sulfate donors. Previous studies have evaluated the extent of sialylation and sulfation in purified secreted mucin from CF patients, 30,31 primary tissue explants, 35,36 and human bronchial xenografts. 33,34 These studies have demonstrated an increase in the extent of sulfation and a decrease in the level of sialylation within large molecular weight purified mucin samples from CF as compared to non-CF.…”

Section: Cftr As An Intracellular Chloride Channelmentioning

confidence: 99%

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Cellular heterogeneity of CFTR expression and function in the lung: implications for gene therapy of cystic fibrosis

Jiang

Engelhardt

1998

Eur J Hum Genet

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Cystic fibrosis (CF) has become a paradigm disorder for the clinical testing of gene therapies in the treatment of inherited disease. In recent years, efforts directed at gene therapy of CF have concentrated on improving gene delivery systems to the airway. Surrogate endpoints for complementation of CFTR dysfunction in the lung have been primarily dependent on correction of chloride transport abnormalities. However, it is now clear that the pathophysiology of CF airways disease is far more complex than can be solely attributed to altered chloride permeability. For example, in addition to functioning as a chloride channel, CFTR also has been implicated in the regulation of other apical membrane conductance pathways through interactions with the amiloride sensitive epithelial sodium channel (ENaC) and the outwardly rectifying chloride channel (ORCC). Superimposed on this functional diversity of CFTR is a highly regulated pattern of CFTR expression in the lung. This heterogeneity occurs at both the level of CFTR protein expression within different cell types in the airway and the anatomical location of these cells in the lung. Potential targets for gene therapy of CF include ciliated, non-ciliated, and goblet cells in the surface airway epithelium as well as submucosal glands within the interstitium of the airways. Each of these distinct cellular compartments may have functionally distinct roles in processes which affect the pathogenesis of CF airways disease, such as fluid and electrolyte balance. However, it is presently unclear which of these cellular targets are most pathophysiologic relevant with regard to gene therapy. Elucidation of the underlying mechanisms of CFTR function in the airway will allow for the rational design of gene therapy approaches for CF lung diseases. This review will provide a summary of the field's current knowledge regarding CFTR functional diversity in the airway and the implications of such diversity for gene therapies of CF lung disease.

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Section: Heterogeneity Of Cftr-expressing Cell Types In the Lungmentioning

confidence: 99%

Section: Cftr As An Intracellular Chloride Channelmentioning

confidence: 99%

Cellular heterogeneity of CFTR expression and function in the lung: implications for gene therapy of cystic fibrosis

Jiang

Engelhardt

1998

Eur J Hum Genet

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“…Serum-type glycoproteins are not only structurally different from mucin-type glycoproteins, but their biosynthesis is entirely different (1 5). Thus these studies do not shed light directly on reported abnormalities of mucins and mucous secretions in CF (37)(38)(39).…”

Section: Discussionmentioning

confidence: 98%

The Carbohydrate Convent of IgG from Patients with Cystic Fibrosis

Margolies

Boat

1983

Pediatr Res

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“…Secretory components released into medium from CF nasal polyp (27) and tracheobronchial explants (28) are similarly oversulfated as assessed by ratios of 3 s~/ 3~(~1~~) incorporation. CF nasal epithelial cells in primary culture oversulfate both secreted and cell surface glycoconjugates.…”

Section: Discussionmentioning

confidence: 99%

Alteration of Sulfation of Glycoconjugates, but Not Sulfate Transport and Intracellular Inorganic Sulfate Content in Cystic Fibrosis Airway Epithelial Cells

et al. 1995

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Mucus Glycoproteins Secreted by Respiratory Epithelial Tissue from Cystic Fibrosis Patients

Cited by 76 publications

References 11 publications

Cellular heterogeneity of CFTR expression and function in the lung: implications for gene therapy of cystic fibrosis

Cellular heterogeneity of CFTR expression and function in the lung: implications for gene therapy of cystic fibrosis

The Carbohydrate Convent of IgG from Patients with Cystic Fibrosis

Alteration of Sulfation of Glycoconjugates, but Not Sulfate Transport and Intracellular Inorganic Sulfate Content in Cystic Fibrosis Airway Epithelial Cells

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