Abstract
Extract: The cell-free medium obtained after 48 hour culture of phytohemagglutinin (PHA)-stimulated leukocytes of three cystic fibrosis (CF) subjects and two carriers, contained a ciliary dyskinesia factor (CDF) detected by a modified rabbit tracheal bioassay. Positive CDF responses began to be observed in supernates of parallel non-stimulated cultures of leukocytes from these same CF affected and carrier individuals by 72 hours of culture. Cell-free media from leukocyte cultures of four normal donors did not produce a positive CDF response with or without PHA over a period of 6 days.
The cell-free medium of long-term lymphoid lines derived from three CF affected and four CF carrier subjects demonstrated a positive CDF response, while media from similar lymphoid lines derived from normal subjects contained no CDF. Distinction between homozygous and heterozygous lymphoid lines was not always possible, although there was a tendency of the homozygous lines to give an earlier CDF response than the heterozygous ones. Addition of rabbit anti-human IgG to the CDF positive cell line supernates resulted in the elimination of their ability to elicit a positive CDF response in each instance.
Speculation: CDF is a manifestation of a normal cellular product which is a small molecule bound to IgG. The resulting complex represents the CDF detected by bioassay. The defect in CF is in the production or release of a factor inhibiting the CDF.
Similar content being viewed by others
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Conover, J., Beratis, N., Conod, E. et al. Studies on Ciliary Dyskinesia Factor in Cystic Fibrosis. II. Short Term Leukocyte Cultures and Long Term Lymphoid Lines. Pediatr Res 7, 224–228 (1973). https://doi.org/10.1203/00006450-197304000-00029
Issue Date:
DOI: https://doi.org/10.1203/00006450-197304000-00029
Keywords
This article is cited by
-
Human IgG subclasses in health and disease (A review)Part I
La Ricerca in Clinica e in Laboratorio (1980)
-
Demonstration of a factor in the serum of homozygotes and heterozygotes for cystic fibrosis by a non-biological technique
Human Genetics (1975)
-
Cystic fibrosis: Demonstration of an abnormality in mucopolysaccharides in cultured lymphoid lines
Biochemical Genetics (1974)