セレブロシドサルフェート活性化因子（ＳＡＰＯＳＩＮ　Ｂ，ＳＡＰ‐１）：多くの局面をもつタンパク質

DOI 参考文献107件
  • Fluharty Arvan L.
    Neurobiochemistry Group, UCLA Mental Retardation Research Center, Department of Psychiatry and Biobehavioral Sciences, School of Medicine, University of California
  • 佐藤 いずみ
    東京工業大学・生命理工学部・生体分子工学科・糖鎖生命科学

書誌事項

タイトル別名
  • The Cerebroside Sulfate Activator(Saposin B, SAP-1): A Many Faceted Protein.
  • A Many Faceted Protein
  • 多くの局面をもつタンパク質

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説明

The Cerebroside Sulfate Activator (CS-Act) is a small protein involved in the catabolism of cerebroside sulfates (sulfatides) and a number of other glycosphingolipids. It is also commonly referred to as SAP-1 or Saposin B. CS-Act is believed to function by binding target lipids, extracting them from membranes or micelles and making them available to water soluble enzymes. The protein probably also plays a role in intermembrane transport of these lipids. The protein is noted for exceptional thermal and proteolytic stability and a multi-amphipathic helix bundle structure stabilized by internal disulfides has been proposed. CS-Act is defective in a rare form of Metachromatic Leukodystrophy and the responsible mutations have been defined. The CS-Act protein is derived from a much larger precursor, Prosaposin, which gives rise to a family of structurally similar proteins believed to have related biochemical activities. The relative abundance of CS-Act, the other Saposins and Prosaposin, as well as the anatomic and developmental profiles of these proteins and their messenger RNA suggests that they may play more profound biological roles than originally envisioned.

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