Am J Physiol. 1990 Dec;259(6 Pt 1):C1010-5.

Genetic complementation in cystic fibrosis pancreatic cells by somatic cell fusion.

Jilling T, Cunningham S, Barker PE, Green MW, Frizzell RA, Kirk KL.

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Gregory Fleming James Cystic Fibrosis Research Center, Department of Physiology and Biophysics, University of Alabama, Birmingham 35294.

Abstract

The biochemical defect that underlies the genetic disorder cystic fibrosis (CF) has been proposed to involve an altered regulation of epithelial Cl- permeability by agents such as adenosine 3',5'-cyclic monophosphate (cAMP). We report here the successful complementation of this functional defect achieved by using the technique of somatic cell fusion to introduce the normal CF allele into mutant cells. CF epithelial cells were fused with transfectant mouse fibroblasts that contain the normal human gene. The resulting heterokaryons were examined for restoration of cAMP-activated Cl- transport using an optical assay of Cl- permeability. Our results provide direct evidence for the involvement of the protein product of the normal CF allele in modulating epithelial Cl- permeability.

PMID:: 1701965; [PubMed - indexed for MEDLINE]

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