1. Combined Mutations of Canalicular Transporter Proteins Cause Severe Intrahepatic Cholestasis of Pregnancy 
Verena Keitel, Christoph Vogt, Dieter Häussinger, Ralf Kubitz 
Gastroenterology 
Volume 131, Issue 2, Pages (August 2006)
DOI: /j.gastro
Copyright © 2006 American Gastroenterological Association Institute Terms and Conditions

2. Figure 1
Liver function tests in the course of the disease. Serum bile salt, transaminase, bilirubin, and γ-glutamyltransferase (γ-GT) levels were monitored during pregnancy. Values are expressed as x-fold of upper normal values, which were 8 μmol/L for bile salts, 31 U/L for aspartate aminotransferase, 35 U/L for alanine aminotransferase, 1.1 mg/dL (18.7 μmol/L) for bilirubin, and 38 U/L for γ-GT. Aspartate aminotransferase and alanine aminotransferase levels became normal, while bile salt levels gradually increased after therapy with ursodeoxycholate (UDCA) was initiated. Spontaneous delivery occurred in the 35th week (large arrows).
Gastroenterology  , DOI: ( /j.gastro )
Copyright © 2006 American Gastroenterological Association Institute Terms and Conditions

3. Figure 2
Topology models of MDR3 and BSEP. Amino acid sequences were derived from NP_ (MDR3) and NP_ (BSEP). Transmembrane domains were estimated according to Tusnády ( Topology models were drawn by the use of the transmembrane protein display software TOPO2 by S. J. Johns ( The mutations found in this patient are within the fifth transmembrane domain (MDR3, S320F) and in the nucleotide binding fold (BSEP, V444A). The relevant sequences from the patient, her mother, and aunt are shown.
Gastroenterology  , DOI: ( /j.gastro )
Copyright © 2006 American Gastroenterological Association Institute Terms and Conditions

4. Figure 3
Immunofluorescence staining of BSEP in liver slices. In control livers, BSEP (A, K24; B, K168; red), MDR3 (green), and the multidrug resistance-related protein 2 (MRP2, green) were all strongly expressed and colocalized at the canalicular membrane. In the patient’s liver MDR3 immunofluorescence (green) was normal, while BSEP immunoreactivity (K24 and K168; red) was markedly reduced. Bars = 10 μm.
Gastroenterology  , DOI: ( /j.gastro )
Copyright © 2006 American Gastroenterological Association Institute Terms and Conditions