Deficiency of arginine esterase in cystic fibrosis of the pancreas: demonstration of the proteolytic nature of the activity

Pediatric Research
G J Rao, H L Nadler

Abstract

Proteolytic activity, defined as the hydrolysis of peptide bonds involving the carboxyl group of L-arginine, in plasma of patients with cystic fibrosis, heterozygotes, and control subjects has been assayed using a fluorometric method with protamine as the substrate and fluorescamine as the reagent. The mean total proteolytic activity in plasma of patients with cystic fibrosis was approximately one-half the mean total activity in control subjects and heterozygotes. The mean proteolytic activity inhibited by soybean trypsin inhibitor in plasma of patients with cystic fibrosis was approximately one-third that of control subjects and heterozygotes. The relationship of arginine esterase activity to proteolytic activity was investigated. The pH optimum and action of reversible and irreversible inhibitors were similar for both activites, suggesting that the arginine esterase activity and proteolytic activity represent similar catalytic entities. These findings are consistent with our hypothesis that the basic defect in cystic fibrosis may reside in the deficieny of a proteolytic enzyme which results in the accumulation of the various cationic macromolecular "factors" described by other investigators in serum of patients with cystic fi...Continue Reading

Citations

Jan 1, 1978·Journal of Inherited Metabolic Disease·L G Dann, K Blau
Jul 1, 1978·Clinica Chimica Acta; International Journal of Clinical Chemistry·G J Guy, J Butterworth
Oct 27, 1982·Clinica Chimica Acta; International Journal of Clinical Chemistry·B TümmlerJ R Riordan
Jan 1, 1983·Annals of the New York Academy of Sciences·M A Bridges, D A Applegarth
Jan 29, 1999·Physiological Reviews·P M Quinton
Aug 9, 1976·Biochemical and Biophysical Research Communications·E ShapiraH L Nadler

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