title: Immunological localization of cystic fibrosis candidate gene products.
abstract: The recent identification of the cystic fibrosis (CF) gene and its putative protein product, the CF transmembrane conductance regulator (CFTR), enabled us to synthesize oligopeptides corresponding with a predicted extracellular domain (position 103-117; peptide A) and a cytoplasmic domain (position 501-515; peptide B) constituting the phenylalanine deletion (F 508) observed in the majority of CF mutations. Immunobiochemical studies with antibodies directed against these peptides revealed the presence of two CFTR candidate proteins (155 and 195 kDa) in various types of epithelial cells. Immunolocalization studies performed on slices of human duodenum showed the strongest expression in the endoplasmic reticulum (RER) of the mucus-producing Goblet cells. Labeling is also demonstrated in the RER and apical membranes of villus and crypt cells, however, to a weaker extent.
authors.affiliation: MGC-Department of Cell Biology and Genetics, Medical Faculty, Erasmus University Rotterdam, The Netherlands.
authors.forename: A T
authors.initials: AT
authors.lastname: Hoogeveen
authors.forename: J
authors.initials: J
authors.lastname: Keulemans
authors.forename: R
authors.initials: R
authors.lastname: Willemsen
authors.forename: B J
authors.initials: BJ
authors.lastname: Scholte
authors.forename: J
authors.initials: J
authors.lastname: Bijman
authors.forename: M J
authors.initials: MJ
authors.lastname: Edixhoven
authors.forename: H R
authors.initials: HR
authors.lastname: De Jonge
authors.forename: H
authors.initials: H
authors.lastname: Galjaard
mesh_terms: D000595:Amino Acid Sequence; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D004721:Endoplasmic Reticulum / Q000378:metabolism; D005455:Fluorescent Antibody Technique; D006801:Humans; D007413:Intestinal Mucosa / Q000378:metabolism; D008565:Membrane Proteins / Q000276:immunology / Q000378:metabolism*; D008969:Molecular Sequence Data; D008970:Molecular Weight; D010455:Peptides / Q000737:chemistry / Q000276:immunology; D011233:Precipitin Tests
chemical_list: C505032:CFTR protein, human; D008565:Membrane Proteins; D010455:Peptides; D019005:Cystic Fibrosis Transmembrane Conductance Regulator