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Research Article Free access | 10.1172/JCI114553

Immunochemical analysis of uridine diphosphate-glucuronosyltransferase in four patients with the Crigler-Najjar syndrome type I.

H H van Es, B G Goldhoorn, M Paul-Abrahamse, R P Elferink, and P L Jansen

Division of Gastroenterology, Academic Medical Center, Amsterdam, The Netherlands.

Find articles by van Es, H. in: PubMed | Google Scholar

Division of Gastroenterology, Academic Medical Center, Amsterdam, The Netherlands.

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Division of Gastroenterology, Academic Medical Center, Amsterdam, The Netherlands.

Find articles by Paul-Abrahamse, M. in: PubMed | Google Scholar

Division of Gastroenterology, Academic Medical Center, Amsterdam, The Netherlands.

Find articles by Elferink, R. in: PubMed | Google Scholar

Division of Gastroenterology, Academic Medical Center, Amsterdam, The Netherlands.

Find articles by Jansen, P. in: PubMed | Google Scholar

Published April 1, 1990 - More info

Published in Volume 85, Issue 4 on April 1, 1990
J Clin Invest. 1990;85(4):1199–1205. https://doi.org/10.1172/JCI114553.
© 1990 The American Society for Clinical Investigation
Published April 1, 1990 - Version history
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Abstract

The functional heterogeneity of uridine diphosphate-glucuronosyltransferase (UDPGT) and its deficiency in human liver were investigated. The monoclonal antibody (MAb) WP1, which inhibits bilirubin and phenol-glucuronidating activity, was used to immunopurify UDPGTs from human liver. Purified UDPGTs were injected into mice to obtain new MAbs. Immunoblotting of microsomes with MAb HEB7 revealed at least three polypeptides in liver (56, 54, and 53 kD) and one in kidney (54 kD). In liver microsomes from four patients (A, B, C, and D) with Crigler-Najjar syndrome type I (CN type I), UDPGT activity towards bilirubin was undetectable (A, B, C, and D) and activity towards phenolic compounds and 5-hydroxytryptamine either reduced (A and B) or normal (C and D). UDPGT activity toward steroids was normal. Immunoblot studies revealed that the monoclonal antibody WP1 recognized two polypeptides (56 and 54 kD) in liver microsomes from patient A and none in patient B. With HEB7 no immunoreactive polypeptides were seen in these two patients. Patient C showed a normal banding pattern and in patient D only the 53-kD band showed decreased intensity. These findings suggest considerable heterogeneity with regard to the expression of UDPGT isoenzymes among CN type I patients.

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