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Scientific paper - review paper
Cystic Fibrosis: Model of Pathogenesis Based on the Apical Membrane Potential
Medicinski glasnik, 12 (2015), 1; 1-6. urn:nbn:hr:239:215205
Kurbel, Beatrica; Rapan, Saša; Kurbel, Sven

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Kurbel, B., Rapan, S. & Kurbel, S. (2015). Cystic Fibrosis: Model of Pathogenesis Based on the Apical Membrane Potential. Medicinski glasnik, 12. (1), 1-6. Retrieved from https://urn.nsk.hr/urn:nbn:hr:239:215205

Kurbel, Beatrica, et al. "Cystic Fibrosis: Model of Pathogenesis Based on the Apical Membrane Potential." Medicinski glasnik, vol. 12, no. 1, 2015, pp. 1-6. https://urn.nsk.hr/urn:nbn:hr:239:215205

Kurbel, Beatrica, Saša Rapan and Sven Kurbel. "Cystic Fibrosis: Model of Pathogenesis Based on the Apical Membrane Potential." Medicinski glasnik 12, no. 1 (2015): 1-6. https://urn.nsk.hr/urn:nbn:hr:239:215205

Kurbel, B., Rapan, S. and Kurbel, S. (2015) 'Cystic Fibrosis: Model of Pathogenesis Based on the Apical Membrane Potential', Medicinski glasnik, 12(1), pp. 1-6. Available at: https://urn.nsk.hr/urn:nbn:hr:239:215205 (Accessed 19 April 2024)

Kurbel B, Rapan S, Kurbel S. Cystic Fibrosis: Model of Pathogenesis Based on the Apical Membrane Potential. Medicinski glasnik [Internet]. 2015 [cited 2024 April 19];12(1):1-6. Available at: https://urn.nsk.hr/urn:nbn:hr:239:215205

B. Kurbel, S. Rapan and S. Kurbel, "Cystic Fibrosis: Model of Pathogenesis Based on the Apical Membrane Potential", Medicinski glasnik, vol. 12, no. 1, pp. 1-6, 2015. [Online]. Available at: https://urn.nsk.hr/urn:nbn:hr:239:215205. [Accessed: 19 April 2024]

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Metadata
Title (english)Cystic Fibrosis: Model of Pathogenesis Based on the Apical Membrane Potential
AuthorBeatrica Kurbel
MBZ: 137831
AuthorSaša Rapan
MBZ: 295480
AuthorSven Kurbel
MBZ: 194496
Author's institution(Clinical hospital center Osijek)
Scientific / art field,
discipline and subdiscipline		BIOMEDICINE AND HEALTHCARE
Clinical Medical Sciences
Internal Medicine
Scientific / art field,
discipline and subdiscipline		BIOMEDICINE AND HEALTHCARE
Basic Medical Sciences
Human Genetics, Genomics and Proteomics
Abstract (english)
A simple model of cystic fibrosis (CF) is proposed, based on the apical membrane (ApM) potential. The ApM of epithelial cells is highly permeable to sodium and activation of CFTRs makes it permeable to chloride. Calculated ApM potentials of cells with activated cystic fibrosis transmembrane conductance regulators (CFTRs) are between the sodium and chloride Nernst values and thus allow rapid absorption of both ions in exocrine glands. In CF patients the potential is near the sodium Nernst value and thus more salt is left in the ducts. Simulation predicts that the sodium driving force increases more than 3.5 times if the ApM permeability for Cl- increases from 5-94% of the sodium permeability. In pancreatic ductal cells basolateral sodium bicarbonate cotransporters (pNBC1) allows influx of bicarbonates with sodium. Bicarbonates are exchanged for intraductal chloride by anion exchanger 1 (AE1) in the ApM. Activated CFTRs let some chloride to leak back to ducts, followed by water that dilutes ductal proteins. Replenished intraductal chloride allows more bicarbonate secretion. In CF patients, pancreatic water and bicarbonate secretion is limited by the intraductal chloride pool.
Keywords (english)
Languageenglish
Publication typeScientific paper - review paper
Publication statusPublished
Peer reviewPeer review - international
Publication versionPublished version
Journal titleMedicinski glasnik
Numberingvol. 12, no. 1, pp. 1-6
p-ISSN1840-2445
e-ISSN1840-0132
URN:NBNurn:nbn:hr:239:215205
Publication2015
Document URLhttps://pdfs.semanticscholar.org/38e7/85aa5627f6160a5064b0beab4e04133bc13c.pdf
Type of resourceText
Access conditionsOpen access
Terms of useLicence In copyright icon
Created on2019-11-06 09:31:01