Cellular localization of the cystic fibrosis transmembrane conductance regulator in mouse intestinal tract

Nadia Ameen, John Alexis, Pedro J Salas

Research output: Contribution to journalArticle

61 Citations (Scopus)

Abstract

The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP and cGMP-regulated chloride channel critical to the regulation of intestinal fluid, chloride, and bicarbonate secretion. In cystic fibrosis (CF), mutations in CFTR result in downregulation of CFTR function and small intestinal obstruction. Unlike the human CF intestine, severe gastrointestinal disease and lethal obstruction is common in transgenic mice deficient in CFTR. The relevance of the physiology of CFTR and pathophysiology of CF in genetically altered mice to that of human CF disease remains incompletely understood. We hypothesized that the expression and distribution of CFTR in mouse intestine may differ from that of human and may contribute to the variation in disease expression between the two species. Using immunocytochemical and immunoblot techniques and well-characterized anti-rodent anti-CFTR antibodies, we examined the cellular distribution of CFTR in the mouse intestinal tract. We identified significant differences in villus distribution for CFTR in the mouse proximal small intestine compared to those previously reported for human and rat. These observations are important to the understanding of CFTR pathophysiology in transgenic CF mouse model systems and bear relevance to the different phenotypic expression of disease in mice compared to human.

Original languageEnglish
Pages (from-to)69-75
Number of pages7
JournalHistochemistry and Cell Biology
Volume114
Issue number1
StatePublished - Aug 19 2000

Fingerprint

cystic fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator
regulators
mice
Cystic Fibrosis
Physiology
intestines
Antibodies
Rats
Intestines
Fluids
Chloride Channels
Gastrointestinal Diseases
Intestinal Obstruction
Bicarbonates
chlorides
Transgenic Mice
Small Intestine
Chlorides
Rodentia

Keywords

  • CFTR
  • Intestine
  • Localization
  • Mouse
  • Protein

ASJC Scopus subject areas

  • Cell Biology
  • Instrumentation

Cite this

Cellular localization of the cystic fibrosis transmembrane conductance regulator in mouse intestinal tract. / Ameen, Nadia; Alexis, John; Salas, Pedro J.

In: Histochemistry and Cell Biology, Vol. 114, No. 1, 19.08.2000, p. 69-75.

Research output: Contribution to journalArticle

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