title: Further evidence for abnormal protein kinase C regulation of macromolecule secretion in fibroblasts from cystic fibrosis patients.
abstract: In comparison to skin fibroblasts from normal subjects, those from patients with cystic fibrosis (CF): (1) bound [20-3H] phorbol 12,13-dibutyrate (PDBu) with a higher affinity Kd = 25.8 vs 12.8 nM respectively) but expressed a similar number of total phorbol ester binding sites (about 2.5 pmol PDBu bound/mg of protein); (2) exhibited a faster and higher response to 4 beta-phorbol 12 beta-myristate 13 alpha-acetate (PMA) for the stimulation of [35S]-labelled glycoconjugate release, but were equally sensitive to the synergistic effect of A23187 on this process; and (3) secreted glycoconjugates with similar [35S]-sulfate and [14C]-leucine to [14C]-glucosamine labelling ratios. Taken together, these results provide further evidence for abnormal protein kinase C (PKC) regulation of macromolecule secretion in CF disease.
authors.affiliation: Laboratoire de Biochimie, Hôpital Sainte-Eugénie, Pierre Bénite, France.
authors.forename: F
authors.initials: F
authors.lastname: Bertrand
authors.forename: B
authors.initials: B
authors.lastname: Hermelin
authors.forename: A
authors.initials: A
authors.lastname: Paul
authors.forename: I
authors.initials: I
authors.lastname: Garcia
authors.forename: J
authors.initials: J
authors.lastname: Capeau
authors.forename: G
authors.initials: G
authors.lastname: Cherqui
authors.forename: J
authors.initials: J
authors.lastname: Picard
mesh_terms: D001665:Binding Sites / Q000502:physiology; D003994:Bucladesine / Q000494:pharmacology; D000001:Calcimycin / Q000494:pharmacology; D002478:Cells, Cultured / Q000187:drug effects; D005576:Colforsin / Q000494:pharmacology; D003550:Cystic Fibrosis / Q000378:metabolism*; D004305:Dose-Response Relationship, Drug; D005347:Fibroblasts / Q000187:drug effects / Q000378:metabolism*; D006001:Glycoconjugates / Q000032:analysis / Q000378:metabolism*; D006801:Humans; D007545:Isoproterenol / Q000494:pharmacology; D015240:Phorbol 12,13-Dibutyrate / Q000378:metabolism*; D011493:Protein Kinase C / Q000032:analysis / Q000378:metabolism*; D012867:Skin; D013755:Tetradecanoylphorbol Acetate / Q000494:pharmacology
chemical_list: D006001:Glycoconjugates; D005576:Colforsin; D015240:Phorbol 12,13-Dibutyrate; D000001:Calcimycin; D003994:Bucladesine; D011493:Protein Kinase C; D007545:Isoproterenol; D013755:Tetradecanoylphorbol Acetate