Fractionation of 4-methylumbelliferyl-ß-galactosidase Activities in Liver in Gargoylism

Abstract

öckerman, P. A. & Hultberg, B. Fractionation of 4-methylumbelliferyl-β-galactosidase Activities in Liver in Gargoylism. Scand. J. clin. Lab. Invest. 22, 199-202, 1968.

The pH-dependency of the 4-methyl-umbelliferyl-β-galactosidase activity was studied in homogenates of liver biopsy specimens from controls and patients with gargoylism. In the controls maximum β-galacto-sidase activity was seen at around pH 4.5 with very little variation, whereas in the gargoylism patients maximum activity was noted near a pH of 5.0-5.5.

By gel filtration on Sephadex G-150 the 4-methyl-umbelliferyl-β-galactosidase activity could be separated into three distinct peaks. The maximum β-galactosidase activity was at around pH 4.0-4.2 for peak I, pH 4.4-4.7 for peak II and pH 5.0-5.5 for peak III. In the controls, peak II was larger than peak I and III. In the patients with gargoylism a different pattern was seen, involving a very marked diminution of peak II, a slight decrease of peak I and no significant change of peak III. These findings may indicate the selective decrease in liver from patients with gargoylism of one or more but not of all enzymes with β-galacto-sidase activity.

Key Words:

• β-galactosidase
• gargoylism
• gel filtration
• liver