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Roles for PMP22 in Schwann cell cholesterol homeostasis in health and disease.

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  • معلومة اضافية
    • المصدر:
      Publisher: Portland Press On The Behalf Of The Biochemical Society Country of Publication: England NLM ID: 7506897 Publication Model: Print Cited Medium: Internet ISSN: 1470-8752 (Electronic) Linking ISSN: 03005127 NLM ISO Abbreviation: Biochem Soc Trans Subsets: MEDLINE
    • بيانات النشر:
      Original Publication: London : Portland Press On The Behalf Of The Biochemical Society
    • الموضوع:
      Cholesterol*/metabolism ; Schwann Cells*/metabolism ; Myelin Proteins*/metabolism ; Myelin Proteins*/genetics ; Homeostasis* ; Charcot-Marie-Tooth Disease*/metabolism ; Charcot-Marie-Tooth Disease*/genetics; Humans ; Animals ; Myelin Sheath/metabolism ; Lipid Metabolism ; ATP Binding Cassette Transporter 1/metabolism ; ATP Binding Cassette Transporter 1/genetics ; Mutation
    • نبذة مختصرة :
      Underexpression, overexpression, and point mutations in peripheral myelin protein 22 (PMP22) cause most cases of Charcot-Marie-Tooth disease (CMTD). While its exact functions remain unclear, PMP22 is clearly essential for formation and maintenance of healthy myelin in the peripheral nervous system. This review explores emerging evidence for roles of PMP22 in cholesterol homeostasis. First, we highlight dysregulation of lipid metabolism in PMP22-based forms of CMTD and recently-discovered interactions between PMP22 and cholesterol biosynthesis machinery. We then examine data that demonstrates PMP22 and cholesterol co-traffic in cells and co-localize in lipid rafts, including how disease-causing PMP22 mutations result in aberrations in cholesterol localization. Finally, we examine roles for interactions between PMP22 and ABCA1 in cholesterol efflux. Together, this emerging body of evidence suggests that PMP22 plays a role in facilitating enhanced cholesterol synthesis and trafficking necessary for production and maintenance of healthy myelin.
      (© 2024 The Author(s).)
    • Grant Information:
      R01 NS095989 United States NS NINDS NIH HHS
    • Contributed Indexing:
      Keywords: Charcot-Marie-Tooth disease; PMP22; cholesterol; myelin; peripheral myelin protein 22; peripheral nervous system
    • الرقم المعرف:
      97C5T2UQ7J (Cholesterol)
      0 (Myelin Proteins)
      0 (PMP22 protein, human)
      0 (ATP Binding Cassette Transporter 1)
    • الموضوع:
      Date Created: 20240709 Date Completed: 20240828 Latest Revision: 20241011
    • الموضوع:
      20241011
    • الرقم المعرف:
      10.1042/BST20231359
    • الرقم المعرف:
      38979632