RPE65-Related Proteins Constitute a Retinoid- and Carotenoid-Metabolizing Protein Family Throughout the Animal and Plant Kingdom

  • Hiroshi Sagara
  • Emiko Suzuki
  • Kazushige Hirosawa
Conference paper
Part of the Keio University International Symposia for Life Sciences and Medicine book series (KEIO, volume 11)


Retinal pigment epithelial (RPE) cells play crucial roles in the maintenance of visual functions. The production and regeneration of 11-cis retinal, which serves as the chromophore of the visual pigment rhodopsin, is one of the most important roles of the RPE cells. To date, several genes that encode the enzymes that may participate in the metabolism of retinoids in RPE cells have been identified [1–3]. However, some of the key enzymes presumed to exist in the RPE cell such as isomerohydrolase have not yet been identified. RPE65 is one of the candidate proteins that may be included in retinoid metabolism in RPE cells.

Key words

RPE65 Retinoid Carotenoid Drosophila DRPE65 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Simon A, Hellman U, Wernstedt C, et al. (1995) The retinal pigment epithelial-specific 11-cis-retinol dehydrogenase belongs to the family of short chain alcohol dehydrogenases. J Biol Chem 270: 1107–1112PubMedCrossRefGoogle Scholar
  2. 2.
    Haeseleer F, Huang J, Lebioda L, et al. (1998) Molecular characterization of a novel short-chain dehydrogenase/reductase that reduces all-trans-retinal. J Biol Chem 273: 21790–21799PubMedCrossRefGoogle Scholar
  3. 3.
    Ruin A, Winston A, Lim Y-H, et al. (1999) Molecular and biochemical characterization of lecithin retinol acyltransferase. J Biol Chem 274: 3834–3841CrossRefGoogle Scholar
  4. 4.
    Sagara H, Hirosawa K (1991) Monoclonal antibodies which recognize endoplasmic reticulum in the retinal pigment epithelium. Exp Eye Res 53: 765–771PubMedCrossRefGoogle Scholar
  5. 5.
    Redmond TM, Hamel CP (2000) Genetic analysis of RPE65: from human disease to mouse model. Methods Enzymol 316: 705–724PubMedCrossRefGoogle Scholar
  6. 6.
    Redmond TM, Yu S, Lee E, et al. (1998) Rpe65 is necessary for production of 11-cisvitamin A in the retinal visual cycle. Nat Genetics 20: 344–351CrossRefGoogle Scholar
  7. 7.
    Lintig J, Vogt K (2000) Filling the gap in vitamin A research. J Cell Biol 257: 11915–11920Google Scholar
  8. 8.
    Lintig J, Dreher A, Kiefer C, et al. (2001) Analysis of the blind Drosophila mutant ninaB identifies the gene encoding the key enzyme for vitamin A formation in vivo. Proc Natl Acad Sci USA 98: 1130–1135CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Tokyo 2003

Authors and Affiliations

  • Hiroshi Sagara
    • 1
  • Emiko Suzuki
    • 1
  • Kazushige Hirosawa
    • 1
  1. 1.Division of Fine Morphology, Department of Basic Medical Sciences, The Institute of Medical ScienceThe University of TokyoMinato-ku, TokyoJapan

Personalised recommendations