Studies on ciliary dyskinesia factor in cystic fibrosis. II. Short term leukocyte cultures and long term lymphoid lines

James H. Conover, Nicholas G. Beratis, Elaine J. Conod, Eugene Ainbender, Kurt Hirschhorn

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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.

Original languageEnglish
Pages (from-to)224-228
Number of pages5
JournalPediatric Research
Issue number4
StatePublished - Apr 1973


  • Ciliary dyskinesia
  • Cystic fibrosis of the pancreas
  • Euglobins
  • Genetic disease


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