Abstract
This report describes the first biosynthetic analysis of the cystic fibrosis transmembrane conductance regulator (CFTR) in freshly excised human rectal biopsies. Expression of functional CFTR was assessed by intestinal current measurement (ICM) prior to biosynthetic studies. Several structural features of CFTR are found to be comparable to those established in CFTR-expressing cell lines. Interestingly, maturation of CFTR increases substantially in tissue incubated at 26 degrees C. Our data provide a solid basis for future studies on the characterisation of CFTR in pathological cases.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Adolescent
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Adult
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Biopsy
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Carbachol / pharmacology
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Child
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Child, Preschool
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Colforsin / pharmacology
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Cysteine / metabolism
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Cystic Fibrosis / pathology
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Cystic Fibrosis Transmembrane Conductance Regulator / analysis*
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Cystic Fibrosis Transmembrane Conductance Regulator / metabolism
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Cystic Fibrosis Transmembrane Conductance Regulator / physiology
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Electrophysiology
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Histamine / metabolism
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Histamine / pharmacology
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Humans
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Immunoprecipitation
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In Vitro Techniques
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Intestinal Mucosa / chemistry
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Intestinal Mucosa / pathology
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Isotope Labeling
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Middle Aged
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Rectum / chemistry*
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Rectum / pathology
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Sulfur Radioisotopes
Substances
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Sulfur Radioisotopes
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Cystic Fibrosis Transmembrane Conductance Regulator
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Colforsin
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Histamine
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Carbachol
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Cysteine