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

Abstract

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-hexosaminidase 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.

Relationships between Serum Markers of Monocyte/Macrophage Activation in Type 1 Gaucher's Disease

“…Neopterin appears to be produced specifically by human activated monocytes/macrophages in response to interferon-γ produced by activated T lymphocytes (14,15). It has also been suggested that β-hexosaminidase (Hex) may also appear in some cases due to macrophage activation (16,17). An increase in its serum/plasma activity in patients with Gaucher's disease has been previously described (18)(19)(20), and may offer a useful approach to its diagnosis and screening (18).…”

Effect of mesocaval interposition shunting and repeated sclerotherapy on blood levels of gastrointestinal regulatory peptides, amino acids, and lysosomal enzymes ‐ a prospective randomised trial

“…The activity of the lysosomal enzymes is increased in liver disease (8,11,12). The reason for this is not known.…”

“…This indicates that serum lysosomal enzymes do not reflect the same liver cell function as the other parameters. The reason for the increased serum lysosomal enzyme activity in liver disease is not known, but several possible explanations have been discussed (8,11,12). We have previously speculated that since the degenerative process in the hepatocytes by itself leads to increased lysoso-ma1 enzyme activity in the hepatocytes, it might 10 explain increased serum lysosomal enzyme activity (8).…”

Role of N-acetyl-beta-d-hexosaminidase in cholesteatoma tissue.

“…Both isoenzymes recognize terminal N-acetylglucosamine and Nacetylgalactosamine, but only isoenzyme A recognizes 6-sulfated residues of these sugars (Winchester, 1996). HEX is active in most tissues and organs such as kidney (Skalova, 2005), spleen (Emiliani et al, 1999), liver (Elsafi et al, 1994), mucous membrane of stomach and intestine (Gil-Martin et al, 1999;Michaels & Hellequist, 2001), cortex (Hammarsund et al, 2004), lung (Baritussio et al, 2001;Minami et al, 1985), epidermal fibroblasts (Ichisaka et al, 1998), placenta (Arciuch et al, 1999) and neoplastic tissues (Bhuvarahamurthy & Govindsamy, 1996;Lerner, 1996;Matsuura et al, 2004). HEX is used as a marker for monitoring the course of sereval diseases like chronic glomerulonephritis (Bazzi et al, 2002), urinaemia (Linko-Lopponen, 1986;Stabellini et al, 2005), arterial hypertension (Perez-Blanco, 1996), Sjogren's syndrom (Sohar et al, 2005), advanced stadium of diabetes (Nakazawa & Tamai, 1991), rheumatoid arthritis (Popko et al, 2005), idiopathic juvenile arthritis (Popko et al, 2003), osteoarthritis (Liu et al, 2004) and for evaluation of kindney function after transplantation (Kotanko et al, 1996).…”