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Clin Liver Dis. 2010 Nov;14(4):619-33. doi: 10.1016/j.cld.2010.08.001.

Trafficking and transporter disorders in pediatric cholestasis.

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  • 1Institute of Liver Studies, King's College Hospital, Denmark Hill, London SE5 9RS, UK. alex.knisely@kcl.ac.uk

Abstract

This article describes the uses of immunostaining in the diagnosis of cholestasis. To immunostain for bile salt export pump (BSEP) and multidrug resistance protein 3 in severe hepatobiliary disease manifest early in life can rapidly identify whether sequencing of ABCB11 or ABCB4 is likely to yield a genetic diagnosis. To immunostain for canalicular ectoenzymes as well as transporters, with transmission electron microscopy, can suggest whether sequencing of ATP8B1 is likely to yield a genetic diagnosis. Demonstrating BSEP expression can direct attention to bile acid synthesis disorders. Immunostaining for multidrug resistance-associated protein 2 serves principally as a control for adequacy of processing.

Copyright © 2010 Elsevier Inc. All rights reserved.

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