An altered hexosaminidase A in the liver affected by Hurler and Hunter syndromes.

Publisher Website
Google Scholar
Abstract
Total hexosaminidase (Hex) in the liver obtained from Hurler and Hunter syndromes displayed approximately 10 times higher acitivty than that in control liver, its thermostability was significantly increased. On DEAE cellulose column chromatography, Hex A in the liver of those patients was eluted at much higher salt concentration than that required for elution of Hex A from control liver. There was no difference in elution patterns of Sephadex G-200 and Concanavalin A-Sepharose column chromatographies between the altered Hex A and control Hex A. The glycosaminoglycans accumulating in the liver of those patients are apparently partly bound to Hex A, and this binding may cause some change of its properties such as ionic charge and thermostability.

This publication has 0 references indexed in Scilit: