Abstract
Liver damage affects the synthesis of proteins and glycoproteins, and alters their posttranslational modification, such as glycosylation changing the serum profile of glycoprotein isoforms. The retention of hydrophobic bile acids in the course of cholestatic liver diseases is a major cause of liver damage in primary biliary cholangitis (PBC). The study objective was to determine the serum profile of transferrin isoforms in primary biliary cholangitis and compare it to transferrin isoforms profile in extrahepatic cholestasis. The study was carried out in 76 patients with PBC and 40 healthy blood donors. Transferrin isoforms were analyzed by the capillary electrophoresis method. The mean relative concentrations of disialotransferrin and trisialotransferrin in PBC were significantly lower than those in the healthy subjects (p < 0.001, p = 0.011; respectively). None of the transferrin isoforms changed according to the disease severity evaluated by the Ludwig scoring system. However, the disease stage affected the activity of alkaline phosphatase (ALP) and γ-glutamyl transferase (GGT), and albumin level (p = 0.002; p = 0.013 and p = 0.005, respectively). Our results indicate that serum profile of transferrin isoforms alters primary biliary cholangitis and differs in comparison to transferrin isoforms profile in extrahepatic cholestasis. The decreased concentrations of lower sialylated isoforms of transferrin (low percentage share in total transferrin level) are not associated with the histological stage of disease.
Highlights
Primary biliary cholangitis (PBC) is a chronic liver disease characterized by damage of small intrahepatic bile ducts resulting in cholestasis, inflammation, and bile acids (BAs) retention [1]
Serum samples were collected from 76 patients with primary biliary cholangitis (PBC) (68 women, 8 men; median age for women: 53 years, range 22–75 years: median age for men: 46 years, range 34–57 years) who were hospitalized at the Department of Gastroenterology, Hepatology and Clinical Oncology of the Centre of Postgraduate Medical Education (Warsaw, Poland)
It is generally accepted that primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by the presence of specific autoantibodies
Summary
Primary biliary cholangitis (PBC) is a chronic liver disease characterized by damage of small intrahepatic bile ducts resulting in cholestasis, inflammation, and bile acids (BAs) retention [1]. BAs may disrupt cell membranes and can promote the generation of reactive oxygen species (ROS) in human liver [3]. ROS generation induces oxidative cell damage causing mitochondrial dysfunction and endoplasmic reticulum stress in isolated rat hepatocytes [4], isolated rat liver mitochondria [5], and isolated human mitochondria [6]. Bile-acid-induced damage in plasma membranes and cell organelle membranes may alter the synthesis of oligosaccharides and subsequently glycoproteins in hepatocytes. The structural alterations involve the aggregation between BAs and lipids in the outer membrane monolayer and the formation of transient holes, which disrupt membrane integrity and resolve of hepatocytes [8]. A key tool for diagnosis PBC and differentiation between PBC, PSC (Primary sclerosing cholangitis) and AIH (Autoimmune hepatitis) is liver biopsy [9,10]
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