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Groups of proteins may form a complex where each protein in this complex has a different role in the overall execution of the complex’s function. Often some of the proteins in the complex can be replaced by a closely related variant to give a complex that contains many of the same components yet is functionally distinct.
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Many proteins form complexes to carry out their functions, making protein-protein interactions (PPIs) essential for an organism's survival. Most PPIs are stabilized by numerous weak noncovalent chemical forces. The physical shape of the interfaces determines the way two proteins interact. Many globular proteins have closely-matching shapes on their surfaces, which form a large number of weak bonds. Additionally, many PPIs occur between two helices or between a surface cleft and a...
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Fatty links between multisystem proteinopathy and small VCP-interacting protein.

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Small VCP-interacting protein (SVIP) acylation is crucial for cell death in multisystem proteinopathy (MSP). Blocking SVIP myristoylation prevents cytotoxicity, suggesting SVIP acylation as a potential therapeutic target for MSP.

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Area of Science:

  • Molecular biology
  • Genetics
  • Cellular biology

Background:

  • Multisystem proteinopathy (MSP) is a rare, dominantly inherited disorder caused by mutations in the valosin-containing protein (VCP) gene.
  • MSP encompasses frontotemporal dementia, inclusion body myopathy, Paget's disease of bone, and amyotrophic lateral sclerosis, with variable patient presentations.
  • VCP's diverse cellular functions are regulated by over 50 co-factors, including small VCP-interacting protein (SVIP).

Purpose of the Study:

  • To investigate the role of SVIP in VCP-mediated cellular dysfunction in MSP.
  • To determine if SVIP acylation influences VCP localization and cellular toxicity.

Main Methods:

  • Investigated SVIP's role in VCP localization to lysosomes.
  • Demonstrated SVIP myristoylation and palmitoylation.
  • Assessed the impact of SVIP acylation on cell death in the presence of a VCP variant.

Main Results:

  • SVIP directs VCP to lysosomes in an acylation-dependent manner.
  • SVIP undergoes myristoylation at Glycine 2 and palmitoylation at Cysteines 4 and 7.
  • SVIP acylation is required for cell death induced by the MSP-associated R155H-VCP variant; blocking myristoylation prevents this toxicity.

Conclusions:

  • SVIP acylation is a critical mediator of VCP-associated cytotoxicity in MSP.
  • Targeting SVIP acylation, particularly myristoylation, may offer a novel therapeutic strategy for MSP.