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The role of ABCR (ABCA4) in photoreceptor cells and Stargardt macular degeneration

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Membrane Transporter Diseases

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Abstract

ATP binding cassette (ABC) transporters are a superfamily of membrane proteins found in virtually all living organisms. They generally function in the active transport of a wide variety of compound s across cell membranes (Higgins 1992; Borst and Elferink 2002). These include amino acids, peptides, ions, metabolites, vitamins, fatty acid derivatives, steroids, organic anions, phospholipids, drugs, and other compounds. ABC transporters typically consist of two membrane domains that provide a pathway for the translocation of a substrate across the membrane and two ATP binding cassettes or nucleo tide binding domains that provide the energy for the substrate transport. These functional domains can exist as individual or multidomain subunits that assemble into an oligomeric complex, or alternatively they all can reside on one large single polypeptide chain (see Chapter 17, Figure 1).

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  1. Biochemistry and Molecular Biology, Faculty of Medicine, University of British Columbia, 2146 Health Sciences Mall, Vancouver, BC, V6T 1Z3, Canada

    Robert S. Molday & Jinhi Ahn

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  1. Robert S. Molday
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  1. Australian National University, Canberra, Australia

    Stefan Bröer

  2. University of Zurich, Zurich, Switzerland

    Carsten A. Wagner

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Molday, R.S., Ahn, J. (2003). The role of ABCR (ABCA4) in photoreceptor cells and Stargardt macular degeneration. In: Bröer, S., Wagner, C.A. (eds) Membrane Transporter Diseases. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-9023-5_20

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  • DOI: https://doi.org/10.1007/978-1-4419-9023-5_20

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