Journal of Hepatology
Volume 53, Issue 2 , Pages 385-387, August 2010

Mutation specific drug therapy for progressive familial or benign recurrent intrahepatic cholestasis: A new tool in a near future?

  • Emmanuel Gonzales
    • ,
  • Emmanuel Jacquemin

      Affiliations

    • Corresponding Author InformationCorresponding author at: Service d’Hépatologie Pédiatrique, Hôpital Bicêtre, 78, rue du Général Leclerc, 94275 Le Kremlin-Bicêtre Cedex, France. Tel.: +33 1 45 21 31 68; fax: +33 1 45 21 28 16.

Received 20 February 2010; received in revised form 18 March 2010; accepted 18 March 2010. published online 07 May 2010.

Abbreviations: PFIC, progressive familial intrahepatic cholestasis, BRIC, benign recurrent intrahepatic cholestasis, LT, liver transplantation, ER, endoplasmic reticulum, ERAD, endoplasmic reticulum associated degradation, 4-PBA, 4-phenylbutyrate, 6-ECDCA, 6-ethyl chenodeoxycholic acid

Keywords: BSEP, MDR3, FIC1, ABCB11, ABCB4, ATP8B1, PFIC, BRIC, Treatment, Nuclear receptor, Chaperone drug, Stop codon skipping

No abstract is available. To read the body of this article, please view the Full Text online.

 

PII: S0168-8278(10)00379-X

doi:10.1016/j.jhep.2010.03.012

Journal of Hepatology
Volume 53, Issue 2 , Pages 385-387, August 2010

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