Abstract
In vitro assays have demonstrated the presence of a “factor” in the serum of patients with cystic fibrosis (CF) which alters mucus production and ciliary action. A more quantitative assay system is needed. Mucociliated “urn cells”, which are found in the coelamic fluid of the marine invertebrate Sipunculus nudus are being used to approach this problem. These cells are easily cultured in their own serum and respond to specific stimuli by secreting mucus tails which vary in rates of response, in quality, and in mean length, depending on the source of the stimulus. This dose-related response can be directly observed in a light microscope, and the length of the mucus tail can be measured. Heated human serum is a known stimulus of mucus production. Thus, human sera from control subjects and patients with CF were tested. Preliminary results show that 18 control sera yielded a mean (± SD) mucus tail length of 1.5 (± 0.21); sera from 12 CF hamozygotes yielded a mean mucus tail length of 6.6 (± 1.3), p < .005. A consistent response was noted when a given serum was retested in the same urn cell suspension, as well as when a given serum was tested in different urn cell suspensions over a time interval of 3 months. Such a population of discrete mucus-producing cells may provide a useful in vitro model for studying the effects of the putative CF factors on mucociliary action.
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Kurlandsky, L., Bang, B., Bang, F. et al. 531 A QUANTITATIVE IN VITRO MODEL FOR THE STUDY OF MUCUS-PRODUCING SUBSTANCES IN CYSTIC FIBROSIS. Pediatr Res 12 (Suppl 4), 452 (1978). https://doi.org/10.1203/00006450-197804001-00536
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DOI: https://doi.org/10.1203/00006450-197804001-00536