期刊
BIOLOGICAL & PHARMACEUTICAL BULLETIN
卷 42, 期 5, 页码 649-665出版社
PHARMACEUTICAL SOC JAPAN
DOI: 10.1248/bpb.b18-00723
关键词
peroxisome; biogenesis; ATP-binding cassette (ABC) transporter; fatty acid metabolism; peroxisome disease; X-linked adrenoleukodystrophy
资金
- Japan Society for the Promotion of Science
- Ministry of Health, Labour and Welfare of Japan
Peroxisomes are indispensable organelles in mammals including humans. They are involved in the beta-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, 1 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.
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