Ciliary dyskinesia factors in cystic fibrosis and asthma.

Using a modified rabbit tracheal bioassay, we have confirmed that serum from 
individuals with bronchial asthma and serum from homozygotes and heterozygotes for 
CF cause ciliary dyskinesia, whereas normal healthy control sera do not. 
To evaluate the specificity of the ciliary dyskinesia activity (CDA) detected, 
we bioassayed serum from eight patients with bronchial asthma. We found 
that all eight asthmatic sera caused a ciliary dyskinesia reaction; however, 
sera from asthmatics generally caused a subsequent ciliostasis (cessation of ciliary 
beating). Concurrent evaluation of all sera for CDA by bioassay and 
for cystic fibrosis protein (CFP) by electrofocusing showed that all CDA-positive 
sera except seven of the eight asthmatic sera were also positive 
for CFP (our specific marker for the CF gene). These two 
finding indicated that the activity detected in patients with asthma and 
other respiratory diseases could be a substance different from a CF-specific 
CDF. To test our hypothesis, we set out to purify the 
CDAs in asthmatic, CF homozygote and OHCF sera. Representative results clearly 
indicate that the asthmatic CDA is found in DEAE-II and Sephadex 
G-200 Fraction II, whereas the CDA found in CF and OHCF 
sera elutes in DEAE-I and Sephadex G-200 Fractions III and IV 
(weak CDA). We conclude that the CDAs in asthma and CF 
are distinct substances. Our findings may facilitate purification of what we 
feel is a CF-specific CDF from CF and OHCF serum. Purification 
is a prerequisite to elucidating the exact nature of this substance 
and its role in the pathophysiology of CF. 