Altered chloride metabolism in cultured cystic fibrosis skin fibroblasts

Journal Article · Fri May 01 00:00:00 EDT 1987 · Proc. Natl. Acad. Sci. U.S.A.; (United States)

DOI:https://doi.org/10.1073/pnas.84.9.3009· OSTI ID:5876125

Mattes, P M; Maloney, P C; Littlefield, J W

An abnormal regulation of chloride permeability has been described for epithelial cells from patients with cystic fibrosis (CF). To learn more about the biochemical basis of this inherited disease, the authors have studied chloride metabolism in cultured CF fibroblasts by comparing the efflux of /sup 36/Cl/sup -/ from matched pairs of CF and normal fibroblasts. The rate constants describing /sup 36/Cl/sup -/ efflux did not differ between the two cell types, but in each of the four pairs tested the amount of /sup 36/Cl/sup -/ contained within CF cells was consistently reduced, by 25-30%, relative to normal cells. Comparisons of cell water content and /sup 22/Na/sup +/ efflux showed no differences between the two cell types, suggesting that overall intracellular chloride concentration is lower than normal in CF fibroblasts. Such data suggest that the CF gene defect is expressed in skin fibroblasts and that this defect may alter the regulation of intracellular Cl/sup -/ concentration, perhaps through changes in Cl/sup -/ permeability.

• Research Organization: Johns Hopkins Univ. School of Medicine, Baltimore, MD
• OSTI ID: 5876125
• Journal Information: Proc. Natl. Acad. Sci. U.S.A.; (United States), Vol. 84:9
• Country of Publication: United States
• Language: English

Related Subjects

62 RADIOLOGY AND NUCLEAR MEDICINE
CHLORINE 36
METABOLISM
HEREDITARY DISEASES
PATHOGENESIS
SODIUM CHLORIDES
CATIONS
CELL MEMBRANES
EPITHELIUM
FIBROBLASTS
MEMBRANE TRANSPORT
PATIENTS
PERMEABILITY
SKIN
SODIUM 22
TIME DEPENDENCE
ALKALI METAL COMPOUNDS
ALKALI METAL ISOTOPES
ANIMAL CELLS
ANIMAL TISSUES
BETA DECAY RADIOISOTOPES
BETA-MINUS DECAY RADIOISOTOPES
BETA-PLUS DECAY RADIOISOTOPES
BODY
CELL CONSTITUENTS
CHARGED PARTICLES
CHLORIDES
CHLORINE COMPOUNDS
CHLORINE ISOTOPES
CONNECTIVE TISSUE CELLS
DISEASES
ELECTRON CAPTURE RADIOISOTOPES
HALIDES
HALOGEN COMPOUNDS
IONS
ISOTOPES
LIGHT NUCLEI
MEMBRANES
NUCLEI
ODD-ODD NUCLEI
ORGANS
RADIOISOTOPES
SODIUM COMPOUNDS
SODIUM ISOTOPES
SOMATIC CELLS
TISSUES
YEARS LIVING RADIOISOTOPES
550601* - Medicine- Unsealed Radionuclides in Diagnostics