{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,16]],"date-time":"2026-07-16T02:21:33Z","timestamp":1784168493005,"version":"3.55.0"},"reference-count":28,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2020,9,8]],"date-time":"2020-09-08T00:00:00Z","timestamp":1599523200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>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 &lt; 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 \u03b3-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.<\/jats:p>","DOI":"10.3390\/jcm9092894","type":"journal-article","created":{"date-parts":[[2020,9,8]],"date-time":"2020-09-08T00:11:51Z","timestamp":1599523911000},"page":"2894","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Changed Profile of Serum Transferrin Isoforms in Primary Biliary Cholangitis"],"prefix":"10.3390","volume":"9","author":[{"given":"Agnieszka","family":"Grytczuk","sequence":"first","affiliation":[{"name":"Department of Laboratory Diagnostics, University Clinical Hospital, 15-540 Bialystok, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2051-1177","authenticated-orcid":false,"given":"Alicja","family":"Bauer","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Biology, Centre of Postgraduate Medical Education, 01-813 Warsaw, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ewa","family":"Gruszewska","sequence":"additional","affiliation":[{"name":"Department of Biochemical Diagnostics, Medical University of Bialystok, 15-269 Bialystok, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bogdan","family":"Cylwik","sequence":"additional","affiliation":[{"name":"Department of Pediatric Laboratory Diagnostics, Medical University of Bialystok, 15-274 Bialystok, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lech","family":"Chrostek","sequence":"additional","affiliation":[{"name":"Department of Biochemical Diagnostics, Medical University of Bialystok, 15-269 Bialystok, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,9,8]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1016\/j.jhep.2017.03.022","article-title":"European Association for the Study of the Liver. EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis","volume":"67","author":"Hirschfield","year":"2017","journal-title":"J. Hepatol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/0306-9877(86)90137-4","article-title":"Bile acid-induced liver toxicity: Relation to the hydrophobichydrophilic balance of bile acids","volume":"19","author":"Attili","year":"1986","journal-title":"Med. Hypotheses"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1016\/0168-8278(90)90114-7","article-title":"Activities of free oxygen radical scavenger enzymes in human liver","volume":"11","author":"Togashi","year":"1990","journal-title":"J. Hepatol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1042\/bj1880321","article-title":"Effects of bile salts on the plasma membranes of isolated rat hepatocytes","volume":"188","author":"Billington","year":"1980","journal-title":"Biochem. J."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1203\/00006450-200104000-00014","article-title":"Role of oxidant stress in the permeability transition induced in rat hepatic mitochondria by hydrophobic bile acids","volume":"49","author":"Sokol","year":"2001","journal-title":"Pediatr. Res."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1097\/01.MPG.0000170600.80640.88","article-title":"Human hepatic mitochondria generate reactive oxygen species and undergo the permeability transition in response to hydrophobic bile acids","volume":"41","author":"Sokol","year":"2005","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"631","DOI":"10.1146\/annurev.bi.54.070185.003215","article-title":"Assembly of asparagine-linked oligosaccharides","volume":"54","author":"Kornfeld","year":"1985","journal-title":"Ann. Rev. Biochem."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"8787","DOI":"10.1021\/bi00424a015","article-title":"Structural changes in vesicle membranes and mixed micelles of various lipid compositions after binding of different bile salts","volume":"27","author":"Schubert","year":"1988","journal-title":"Biochemistry"},{"key":"ref_9","first-page":"1257","article-title":"Primary biliary cirrhosis","volume":"60","author":"Scheuer","year":"1967","journal-title":"Proc. R. Soc. Med."},{"key":"ref_10","first-page":"43","article-title":"Surgical pathology of the syndrome of primary sclerosing cholangitis","volume":"13","author":"Ludwig","year":"1989","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"448","DOI":"10.1056\/NEJM199902113400607","article-title":"Acute-phase proteins and other systemic responses to 17 inflammation","volume":"340","author":"Gabay","year":"1999","journal-title":"N. Engl. J. Med."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/0009-8981(87)90157-4","article-title":"Human serum sialo transferrins in diseases","volume":"165","author":"Koster","year":"1987","journal-title":"Clin. Chim. Acta"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1253","DOI":"10.1172\/JCI117775","article-title":"Kinetic modeling and math-ematical analysis indicate that acute phase gene expression in Hep 3B cells is regulated by both transcriptional and posttranscriptional mechanisms","volume":"95","author":"Jiang","year":"1995","journal-title":"J. Clin. Investig."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1111\/j.1749-6632.1995.tb32336.x","article-title":"Interleukin-6-type cytokine-induced changes in acute phase protein glycosylation","volume":"762","author":"Mackiewicz","year":"1995","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1084\/jem.177.3.657","article-title":"Inflammation-induced expression of sialyl Lewis X-containing glycan structures on alpha 1-acid glycoprotein (orosomucoid) in human sera","volume":"177","author":"Anbergen","year":"1993","journal-title":"J. Exp. Med."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1136\/gut.31.4.431","article-title":"Agalactosyl IgG in inflammatory bowel disease: Correlation with C-reactive protein","volume":"31","author":"Dube","year":"1990","journal-title":"Gut"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"291","DOI":"10.3343\/alm.2019.39.3.291","article-title":"Detection of Autoantibodies Against Nucleoporin p62 in Sera of Patients with Primary Biliary Cholangitis","volume":"39","author":"Bauer","year":"2019","journal-title":"Ann. Lab. Med."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"3525","DOI":"10.4049\/jimmunol.138.10.3525","article-title":"Identification and specificity of a cDNA encoding the 70 kd mitochondrial antigen recognized in primary biliary cirrhosis","volume":"138","author":"Gershwin","year":"1987","journal-title":"J. Immunol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1261","DOI":"10.1056\/NEJMra043898","article-title":"Primary biliary cirrhosis","volume":"353","author":"Kaplan","year":"2005","journal-title":"N. Engl. J. Med."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1007\/s12016-017-8656-x","article-title":"Geoepidemiology of Primary Biliary Cholangitis: Lessons from Switzerland","volume":"54","author":"Stirnimann","year":"2018","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1016\/S0168-8278(99)80098-1","article-title":"Clinical and biochemical expression of the histopathological lesions of primary biliary cirrhosis. UDCA-PBC Group","volume":"30","author":"Poupon","year":"1999","journal-title":"J. Hepatol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1002\/hep.22906","article-title":"American Association for Study of Liver Diseases. Primary biliary cirrhosis","volume":"50","author":"Lindor","year":"2009","journal-title":"Hepatology"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1172\/JCI4765","article-title":"Toxic bile salts induce rodent hepatocyte apoptosis via direct activation of Fas","volume":"103","author":"Faubion","year":"1999","journal-title":"J. Clin. Investig."},{"key":"ref_24","unstructured":"Scheuer, P.J. (1980). Liver Biopsy Interpretation, Balliere Tindall."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1131","DOI":"10.1016\/j.clinbiochem.2017.06.013","article-title":"The transferrin isoforms in chronic hepatitis","volume":"50","author":"Gruszewska","year":"2017","journal-title":"Clin. Biochem."},{"key":"ref_26","first-page":"286","article-title":"Changes in transferrin isoforms in pancreatic cancer","volume":"46","author":"Gruszewska","year":"2016","journal-title":"Ann. Clin. Lab. Sci."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1097\/RHU.0000000000000808","article-title":"The profile of serum transferrin isoforms in rheumatoid arthritis","volume":"25","author":"Gudowska","year":"2019","journal-title":"JCR J. Clin. Rheumatol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"349","DOI":"10.7754\/Clin.Lab.2016.160636","article-title":"Changed profile of serum transferrin isoforms in liver diseases","volume":"63","author":"Gudowska","year":"2017","journal-title":"Clin. Lab."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/9\/9\/2894\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T10:07:45Z","timestamp":1760177265000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/9\/9\/2894"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,9,8]]},"references-count":28,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2020,9]]}},"alternative-id":["jcm9092894"],"URL":"https:\/\/doi.org\/10.3390\/jcm9092894","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,9,8]]}}}