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  • 标题:Biogenesis and Function of Peroxisomes in Human Disease with a Focus on the ABC Transporter
  • 本地全文:下载
  • 作者:Tsuneo Imanaka
  • 期刊名称:Biological and Pharmaceutical Bulletin
  • 印刷版ISSN:0918-6158
  • 电子版ISSN:1347-5215
  • 出版年度:2019
  • 卷号:42
  • 期号:5
  • 页码:649-665
  • DOI:10.1248/bpb.b18-00723
  • 出版社:The Pharmaceutical Society of Japan
  • 摘要:

    Peroxisomes are indispensable organelles in mammals including humans. They are involved in the β-oxidation of very long chain fatty acids, and the synthesis of ether phospholipids and bile acids. Pre-peroxisomes bud from endoplasmic reticulum and peroxisomal membrane and matrix proteins are imported to the pre-peroxisomes. Then, matured peroxisomes grow by division. Impairment of the biogenesis and function of peroxisomes results in severe diseases. Since I first undertook peroxisome research in Prof. de Duve’s laboratory at Rockefeller University in 1985, I have continuously studied peroxisomes for more than 30 years, with a particular focus on the ATP-binding cassette (ABC) transporters. Here, I review the history of peroxisome research, the biogenesis and function of peroxisomes, and peroxisome disease including X-linked adrenoleukodystrophy. The review includes the targeting and function of the ABC transporter subfamily D.

  • 关键词:peroxisome;biogenesis;ATP-binding cassette (ABC) transporter;fatty acid metabolism;peroxisome disease;X-linked adrenoleukodystrophy
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