Further studies on lymphocyte beta-glucuronidase abnormality in cystic fibrosis. Possible relations to carboxypeptidase B-like activity.

The presence in the serum of both cystic fibrosis (CF) homozygotes and 
heterozygotes of a factor inhibiting the response of lymphocyte lysosome beta-glucuronidase 
activity to in vitro phytohaemagglutinin (PHA) stimulation is confirmed. Studying lymphocyte 
beta- glucuronidase activity on PHA stimulation represents a way to confirm 
CF diagnosis and to screen CF heterozygotes. For technical complexity, however, 
the method cannot be used for mass screening, but it can 
be useful for confirming the diagnosis in suspected cases. Relationships between 
serum factor inhibiting the effect of PHA on beta-glucuronidase, ciliary dyskinesia 
factor and carboxypeptidase B- like activity are discussed. 