Abstract
Previous studies have indicated that α-D-glucosidase activity is increased and exhibits abnormal properties in tissues from patients with cystic fibrosis (CF). In the present investigation serum α-D-glucosidase from patients with CF and from patients with chronic pulmonary disease (e.g. asthma and bronchiectasis) has been studied to determine the specificity of the α-D-glucosidase alteration(s) in CF sera. Both groups of patients have elevated α-D-glucosidase activity levels and similarly abnormal isoelectric focusing profiles with significantly less activity associated with acidic enzymatic forms (i.e. having isoelectric points below 4.8). These results suggest that the abnormalities of CF serum α-D-glucosidase may be secondary to chronic pulmonary disease and emphasize the importance of including appropriate pathological controls in biochemical studies on CF.
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Alhadeff, J., Pollack, B., Hopfer, R. et al. Serum Neutral α-D-Glucosidase from Patients with Cystic Fibrosis and Chronic Pulmonary Disease. Pediatr Res 19, 171–174 (1985). https://doi.org/10.1203/00006450-198502000-00004
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DOI: https://doi.org/10.1203/00006450-198502000-00004