Peroxisome Biogenesis and Its Dysfunction

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  • Fujiki Yukio
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School

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  • 細胞内の膜オルガネラの創成機構　　ペルオキシソームの形成機構とその破綻
  • ペルオキシソームの形成機構とその破綻
  • ペルオキシソーム ノ ケイセイ キコウ ト ソノ ハタン

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Abstract

To investigate peroxisome biogenesis and human peroxisome biogenesis disorders, more than a dozen complementation groups of Chinese hamster ovary (CHO) cell mutants defective in peroxisome assembly have been successfully isolated and established as a model system. Moreover, successful cloning of PEX genes encoding peroxisome assembly factors, termed peroxins, by taking advantage of rapid functional complementation assay of CHO cell mutants invaluably contributed to the accomplishment of isolation of pathogenic genes responsible for peroxisome biogenesis diseases. Molecular mechanisms of peroxisome assembly are currently investigated by making use of such mammalian cell mutants. <br>In contrast to molecular mechanisms underlying peroxisomal import of matrix proteins, those involving the transport of membrane proteins and membrane assembly remained elusive. Three peroxins, Pex3p, Pex16p, and Pex19p, were cloned as essential factors for membrane assembly in several species including humans. Recently, two distinct import pathways of peroxisomal membrane proteins (PMPs) have been proposed. Pex19p functions as a chaperone in forming its complexes with newly synthesized PMPs excluding Pex3p in the cytosol and transporting them to peroxosmes by docking to Pex3p. Thereby, PMPs are integrated and Pex19 shuttles back to the cytosol.

Journal

  • MEMBRANE

    MEMBRANE 33 (1), 9-16, 2008

    THE MEMBRANE SOCIETY OF JAPAN

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