ISSN:
1573-4935
Keywords:
cystic fibrosis
;
protein kinase C
;
glycoconjugate secretion
;
phorbol esters
Source:
Springer Online Journal Archives 1860-2000
Topics:
Biology
,
Chemistry and Pharmacology
Notes:
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 4β-phorbol 12β-myristate 13α-acetate (PMA) for the stimulation of [35S]-labelled glycoconjutate 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.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF01116617
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