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The AAA+ ATPase/ubiquitin ligase mysterin stabilizes cytoplasmic lipid droplets

Munechika Sugihara1, Daisuke Morito2,3, Shiori Ainuki1

  • 1Faculty of Life Sciences, Kyoto Sangyo University, Kyoto, Japan.

The Journal of Cell Biology
|February 2, 2019
PubMed

Insights

Mysterin (RNF213) protein regulates lipid droplets (LDs) by eliminating adipose triglyceride lipase (ATGL). MMD-related mutations impair this fat-stabilizing activity, linking moyamoya disease to fat metabolism.

Area of Science:

  • Biochemistry
  • Cell Biology
  • Genetics

Background:

  • Mysterin (RNF213) is an intracellular protein linked to moyamoya disease (MMD).
  • Its biological functions, despite known ubiquitin ligase and ATPase activities, remain unclear.
  • Lipid droplets (LDs) are organelles central to neutral lipid storage.

Purpose of the Study:

  • To investigate the biological role of mysterin (RNF213).
  • To determine mysterin's effect on lipid droplet (LD) abundance and regulation.
  • To explore the connection between MMD pathogenesis and fat metabolism.

Main Methods:

  • Cellular localization studies to determine mysterin's target organelles.
  • Biochemical assays to assess mysterin's enzymatic activities and interactions.
  • Analysis of MMD-related mutations in mysterin's functional domains.

Main Results:

  • Mysterin (RNF213) is targeted to lipid droplets (LDs), significantly increasing their abundance.
  • Mysterin promotes LD accumulation by eliminating adipose triglyceride lipase (ATGL) from LDs.
  • Both ubiquitin ligase and ATPase activities are crucial for mysterin's LD targeting and fat-stabilizing function.
  • MMD-associated mutations in the ubiquitin ligase domain impair mysterin's ability to stabilize LDs.

Conclusions:

  • Mysterin (RNF213) is identified as a novel regulator of cytoplasmic lipid droplets (LDs).
  • The findings suggest a potential link between moyamoya disease (MMD) pathogenesis and cellular fat metabolism.
  • Mysterin's role in LD regulation offers new insights into MMD etiology.

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