Assessment of the CFTR and ENaC association

Bakhrom K. Berdiev, Yawar J. Qadri, Dale J. Benos

Research output: Contribution to journalArticle

73 Citations (Scopus)

Abstract

Cystic fibrosis (CF) is one of the most common lethal genetic disorders. It results primarily from mutations in the cystic fibrosis transmembrane conductance regulator (cftr) gene. These mutations cause inadequate functioning of CFTR, which in turn leads to the severe disruption of transport function in several epithelia across various organs. Affected organs include the sweat glands, the intestine, and the reproductive system, with the most devastating consequences due to the effects of the disease on airways. Despite aggressive treatment, gradual lung failure is the major life limiting factor in patients with CF. Understanding of the exact manner by which defects in the CFTR lead to lung failure is thus critical. In the CF airway, decreased chloride secretion and increased salt absorption is observed. The decreased chloride secretion appears to be a direct consequence of defective CFTR; however, the increased salt absorption is believed to result from the failure of CFTR to restrict salt absorption through a sodium channel named the epithelial Na + channel, ENaC. The mechanism by which CFTR modulates the function of ENaC proteins is still obscure and somewhat controversial. In this short review we will focus on recent findings of a possible direct CFTR and ENaC association.

Original languageEnglish
Pages (from-to)123-227
Number of pages105
JournalMolecular BioSystems
Volume5
Issue number2
DOIs
Publication statusPublished - 2009
Externally publishedYes

Fingerprint

Cystic Fibrosis
Salts
Chlorides
Epithelial Sodium Channels
Cystic Fibrosis Transmembrane Conductance Regulator
Sweat Glands
Lung
Mutation
Inborn Genetic Diseases
Sodium Channels
Regulator Genes
Intestines
Epithelium
Proteins
Therapeutics

ASJC Scopus subject areas

  • Biotechnology
  • Molecular Biology

Cite this

Berdiev, B. K., Qadri, Y. J., & Benos, D. J. (2009). Assessment of the CFTR and ENaC association. Molecular BioSystems, 5(2), 123-227. https://doi.org/10.1039/b810471a

Assessment of the CFTR and ENaC association. / Berdiev, Bakhrom K.; Qadri, Yawar J.; Benos, Dale J.

In: Molecular BioSystems, Vol. 5, No. 2, 2009, p. 123-227.

Research output: Contribution to journalArticle

Berdiev, BK, Qadri, YJ & Benos, DJ 2009, 'Assessment of the CFTR and ENaC association', Molecular BioSystems, vol. 5, no. 2, pp. 123-227. https://doi.org/10.1039/b810471a
Berdiev, Bakhrom K. ; Qadri, Yawar J. ; Benos, Dale J. / Assessment of the CFTR and ENaC association. In: Molecular BioSystems. 2009 ; Vol. 5, No. 2. pp. 123-227.
@article{63f7639ae98b40e49a321239000041de,
title = "Assessment of the CFTR and ENaC association",
abstract = "Cystic fibrosis (CF) is one of the most common lethal genetic disorders. It results primarily from mutations in the cystic fibrosis transmembrane conductance regulator (cftr) gene. These mutations cause inadequate functioning of CFTR, which in turn leads to the severe disruption of transport function in several epithelia across various organs. Affected organs include the sweat glands, the intestine, and the reproductive system, with the most devastating consequences due to the effects of the disease on airways. Despite aggressive treatment, gradual lung failure is the major life limiting factor in patients with CF. Understanding of the exact manner by which defects in the CFTR lead to lung failure is thus critical. In the CF airway, decreased chloride secretion and increased salt absorption is observed. The decreased chloride secretion appears to be a direct consequence of defective CFTR; however, the increased salt absorption is believed to result from the failure of CFTR to restrict salt absorption through a sodium channel named the epithelial Na + channel, ENaC. The mechanism by which CFTR modulates the function of ENaC proteins is still obscure and somewhat controversial. In this short review we will focus on recent findings of a possible direct CFTR and ENaC association.",
author = "Berdiev, {Bakhrom K.} and Qadri, {Yawar J.} and Benos, {Dale J.}",
year = "2009",
doi = "10.1039/b810471a",
language = "English",
volume = "5",
pages = "123--227",
journal = "Molecular BioSystems",
issn = "1742-206X",
publisher = "Royal Society of Chemistry",
number = "2",

}

TY - JOUR

T1 - Assessment of the CFTR and ENaC association

AU - Berdiev, Bakhrom K.

AU - Qadri, Yawar J.

AU - Benos, Dale J.

PY - 2009

Y1 - 2009

N2 - Cystic fibrosis (CF) is one of the most common lethal genetic disorders. It results primarily from mutations in the cystic fibrosis transmembrane conductance regulator (cftr) gene. These mutations cause inadequate functioning of CFTR, which in turn leads to the severe disruption of transport function in several epithelia across various organs. Affected organs include the sweat glands, the intestine, and the reproductive system, with the most devastating consequences due to the effects of the disease on airways. Despite aggressive treatment, gradual lung failure is the major life limiting factor in patients with CF. Understanding of the exact manner by which defects in the CFTR lead to lung failure is thus critical. In the CF airway, decreased chloride secretion and increased salt absorption is observed. The decreased chloride secretion appears to be a direct consequence of defective CFTR; however, the increased salt absorption is believed to result from the failure of CFTR to restrict salt absorption through a sodium channel named the epithelial Na + channel, ENaC. The mechanism by which CFTR modulates the function of ENaC proteins is still obscure and somewhat controversial. In this short review we will focus on recent findings of a possible direct CFTR and ENaC association.

AB - Cystic fibrosis (CF) is one of the most common lethal genetic disorders. It results primarily from mutations in the cystic fibrosis transmembrane conductance regulator (cftr) gene. These mutations cause inadequate functioning of CFTR, which in turn leads to the severe disruption of transport function in several epithelia across various organs. Affected organs include the sweat glands, the intestine, and the reproductive system, with the most devastating consequences due to the effects of the disease on airways. Despite aggressive treatment, gradual lung failure is the major life limiting factor in patients with CF. Understanding of the exact manner by which defects in the CFTR lead to lung failure is thus critical. In the CF airway, decreased chloride secretion and increased salt absorption is observed. The decreased chloride secretion appears to be a direct consequence of defective CFTR; however, the increased salt absorption is believed to result from the failure of CFTR to restrict salt absorption through a sodium channel named the epithelial Na + channel, ENaC. The mechanism by which CFTR modulates the function of ENaC proteins is still obscure and somewhat controversial. In this short review we will focus on recent findings of a possible direct CFTR and ENaC association.

UR - http://www.scopus.com/inward/record.url?scp=58549087743&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=58549087743&partnerID=8YFLogxK

U2 - 10.1039/b810471a

DO - 10.1039/b810471a

M3 - Article

VL - 5

SP - 123

EP - 227

JO - Molecular BioSystems

JF - Molecular BioSystems

SN - 1742-206X

IS - 2

ER -