Journal
CLINICS IN LIVER DISEASE
Volume 14, Issue 4, Pages 619-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.cld.2010.08.001
Keywords
Pediatric cholestasis; Bile salt export pump; Multidrug resistance protein; Genetic diagnosis; Cell polarity
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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
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