Lysosomes and Human Liver Disease: A Biochemical and Immunohistochemical Study of ß-Hexosaminidase

Summary: Liver biopsies from 88 patients with different liver diseases were studied for ß-hexosaminidase activity. Liver specimens with normal light microscopical morphology showed no immunohistochemical reactivity for ß-hexosaminidase. Increased reactions were noted, mainly in hepatocytes, in biopsies from the patients with different liver diseases. A very large interindividual Variation of biochemical liver ß-hexosamindase activity occurred even within the same diagnostic group, and no group of patients showed any significant increase of liver ß-hexosaminid-ase activity compared with the patients with normal liver histology. Livers with positive immunohistochemistry showed increased biochemical values for ß-hexosaminidase. In patients with cholestasis due to alcohol abuse, the immunohistochemical reaetion was intense and the biochemical ß-hexosaminidase activity was significantly increased compared with non-alcöholic cholestatic cases. Furthermore, blood samples were taken from 50 patients at the same time äs the liver biopsies. These patients showed significantly increased serum ß-hexosaminidase activity compared with normal coiitrols, but no correlation was found between ß-hexosaminidase activities in the liver and serum of these patients. Introduction ß-hexosaminidase in hepatocytes and to some extent in T.,.,.,-, Kupffer cells in various types of liver disease (6).Lysosomal storage diseases are diagnosed by absence or low activity of a specific enzyme in plasma and/or in All lysosomal enzymes are initially synthesized in th