Carboxypeptidase B activity of cultured skin fibroblasts and relationship to cystic fibrosis

Clinica Chimica Acta; International Journal of Clinical Chemistry
J Butterworth, J J Duncan


Enzyme activity against hippuryl-L-arginine was studied in cultured skin fibroblasts from controls and cystic fibrosis patients. The enzyme had a lysosomal distribution and an acid optimum of pH 4.5 with little or no activity present above pH 7.0. Dithiothreitol was required for full activity and the kinetics of thiol activation were different for the control and cystic fibrosis enzyme. The properties and lysosomal distribution of the enzyme indicated that it was a carboxypeptidase B. Substrate affinity, thermolability, pH stability, the fall and rise in activity with subculture, the cyclical pattern of activity through serial passage and the level of activity were similar for the control and cystic fibrosis enzyme.


Oct 1, 1973·Proceedings of the Society for Experimental Biology and Medicine·B L ShapiroW J Warwick
Jul 1, 1962·Biochemical Pharmacology·E G ERDOS, E M SLOANE


Nov 20, 1980·Clinica Chimica Acta; International Journal of Clinical Chemistry·J Butterworth, J J Duncan
Jun 16, 1982·Clinica Chimica Acta; International Journal of Clinical Chemistry·J Butterworth, D Priestman
Jan 31, 1984·Clinica Chimica Acta; International Journal of Clinical Chemistry·C H BuysH Smith

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