Literature DB >> 23284077

Status of fluid and electrolyte absorption in cystic fibrosis.

M M Reddy1, M Jackson Stutts.   

Abstract

Salt and fluid absorption is a shared function of many of the body's epithelia, but its use is highly adapted to the varied physiological roles of epithelia-lined organs. These functions vary from control of hydration of outward-facing epithelial surfaces to conservation and regulation of total body volume. In the most general context, salt and fluid absorption is driven by active Na(+) absorption. Cl(-) is absorbed passively through various available paths in response to the electrical driving force that results from active Na(+) absorption. Absorption of salt creates a concentration gradient that causes water to be absorbed passively, provided the epithelium is water permeable. Key differences notwithstanding, the transport elements used for salt and fluid absorption are broadly similar in diverse epithelia, but the regulation of these elements enables salt absorption to be tailored to very different physiological needs. Here we focus on salt absorption by exocrine glands and airway epithelia. In cystic fibrosis, salt and fluid absorption by gland duct epithelia is effectively prevented by the loss of cystic fibrosis transmembrane conductance regulator (CFTR). In airway epithelia, salt and fluid absorption persists, in the absence of CFTR-mediated Cl(-) secretion. The contrast of these tissue-specific changes in CF tissues is illustrative of how salt and fluid absorption is differentially regulated to accomplish tissue-specific physiological objectives.

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Year:  2013        PMID: 23284077      PMCID: PMC3530040          DOI: 10.1101/cshperspect.a009555

Source DB:  PubMed          Journal:  Cold Spring Harb Perspect Med        ISSN: 2157-1422            Impact factor:   6.915


  155 in total

Review 1.  Mechanisms of ENaC regulation and clinical implications.

Authors:  Vivek Bhalla; Kenneth R Hallows
Journal:  J Am Soc Nephrol       Date:  2008-08-27       Impact factor: 10.121

2.  Influence of abnormal Cl- impermeability on sweating in cystic fibrosis.

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Journal:  Am J Physiol       Date:  1984-07

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Review 4.  Insight toward epithelial Na+ channel mechanism revealed by the acid-sensing ion channel 1 structure.

Authors:  James D Stockand; Alexander Staruschenko; Oleh Pochynyuk; Rachell E Booth; Dee U Silverthorn
Journal:  IUBMB Life       Date:  2008-09       Impact factor: 3.885

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Journal:  J Clin Invest       Date:  1984-06       Impact factor: 14.808

6.  Effect of cytosolic pH on epithelial Na+ channel in normal and cystic fibrosis sweat ducts.

Authors:  M M Reddy; X F Wang; P M Quinton
Journal:  J Membr Biol       Date:  2008-10-21       Impact factor: 1.843

7.  Effects of aldosterone and dexamethasone on apical membrane properties and Na-transport of rabbit distal colon in vitro.

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Journal:  Pflugers Arch       Date:  1985-02       Impact factor: 3.657

8.  Activity of the p110-alpha subunit of phosphatidylinositol-3-kinase is required for activation of epithelial sodium transport.

Authors:  Jian Wang; Zachary A Knight; Dorothea Fiedler; Olusegun Williams; Kevan M Shokat; David Pearce
Journal:  Am J Physiol Renal Physiol       Date:  2008-07-23

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Authors:  L L Frase; A D Strickland; G W Kachel; G J Krejs
Journal:  Gastroenterology       Date:  1985-02       Impact factor: 22.682

10.  Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid.

Authors:  J J Smith; S M Travis; E P Greenberg; M J Welsh
Journal:  Cell       Date:  1996-04-19       Impact factor: 41.582

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  6 in total

1.  Truncating Mutations in the Adhesion G Protein-Coupled Receptor G2 Gene ADGRG2 Cause an X-Linked Congenital Bilateral Absence of Vas Deferens.

Authors:  Olivier Patat; Adrien Pagin; Aurore Siegfried; Valérie Mitchell; Nicolas Chassaing; Stanislas Faguer; Laetitia Monteil; Véronique Gaston; Louis Bujan; Monique Courtade-Saïdi; François Marcelli; Guy Lalau; Jean-Marc Rigot; Roger Mieusset; Eric Bieth
Journal:  Am J Hum Genet       Date:  2016-07-28       Impact factor: 11.025

Review 2.  Regulation of epithelial ion transport in exocrine glands by store-operated Ca2+ entry.

Authors:  Axel R Concepcion; Stefan Feske
Journal:  Cell Calcium       Date:  2016-12-21       Impact factor: 6.817

Review 3.  Claudins: Gatekeepers of lung epithelial function.

Authors:  Barbara Schlingmann; Samuel A Molina; Michael Koval
Journal:  Semin Cell Dev Biol       Date:  2015-05-04       Impact factor: 7.727

4.  Recurrent episodes of unexplained hypoelectrolytaemia of a rare cause in a young Saudi girl.

Authors:  Mohsen Suliaman Al-Atawi; Sulaiman Abdullah Al-Queflie; Hamad Abdullah Al-Sadoon
Journal:  BMJ Case Rep       Date:  2015-10-22

Review 5.  Epithelial sodium channel (ENaC) family: Phylogeny, structure-function, tissue distribution, and associated inherited diseases.

Authors:  Israel Hanukoglu; Aaron Hanukoglu
Journal:  Gene       Date:  2016-01-07       Impact factor: 3.688

6.  The effects of exercise and passive heating on the sweat glands ion reabsorption rates.

Authors:  Nicola Gerrett; Tatsuro Amano; Yoshimitsu Inoue; George Havenith; Narihiko Kondo
Journal:  Physiol Rep       Date:  2018-03
  6 in total

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