Trafficking and Transporter Disorders in Pediatric Cholestasis

AS Knisely, Paul Gissen

Research output: Contribution to journalArticle

12 Citations (Scopus)


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
Original languageEnglish
Pages (from-to)619-+
JournalClinics in Liver Disease
Issue number4
Publication statusPublished - 1 Nov 2010


  • Multidrug resistance protein
  • Genetic diagnosis
  • Pediatric cholestasis
  • Cell polarity
  • Bile salt export pump


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