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Two Liver Transporters, ABCB11 and ABCC6; Novel Therapeutic Approaches in the Related Disorders

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Abstract

The bile salt export pump, ABCB11, selectively expressed in the hepatocytes, mediates the transport of monovalent bile salts into the bile. Mutations and polymorphisms in ABCB11 may lead to various forms of cholestatic liver diseases ranging from milder symptoms to lethal conditions. Similarly, inborn errors in ABCC6, another hepatic ABC transporter, may result in diverse conditions, which are characterized by soft tissue mineralization. In this chapter we review the recent knowledge on the structure, transport function, tissue distribution, transcriptional and posttranscriptional regulation, as well as the physiological and pathophysiological role of these medically important hepatic transporters. Substantial data on the pathomechanisms of the diseases associated with the dysfunction of these hepatic transporters have been accumulated in the recent years. This knowledge allows novel, mutation-specific therapeutic interventions opening a new perspective for the treatment of these diseases.

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Acknowledgements

The authors are grateful for the support of the Momentum Program of the Hungarian Academy of Sciences (LP 2012–025) to L.H. and for the OTKA Hungarian Research Grants (K104227 and NN114136) to A.V.

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  1. Institute of Enzymology, Research Centre for Natural Sciences, Hungarian Academy of Sciences, Magyar Tudósok Körútja 2, Budapest, 1117, Hungary

    András Váradi & László Homolya

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  1. András Váradi
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  2. László Homolya
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Correspondence to László Homolya .

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  1. School of Life Sciences, University of Technology, Sydney, Australia

    Anthony M. George

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Váradi, A., Homolya, L. (2016). Two Liver Transporters, ABCB11 and ABCC6; Novel Therapeutic Approaches in the Related Disorders. In: George, A. (eds) ABC Transporters - 40 Years on. Springer, Cham. https://doi.org/10.1007/978-3-319-23476-2_15

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