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Cited by 58Genetic Neurodegenerative DiseasesHeat shock proteins researchUbiquitin and proteasome pathways

Valosin containing protein (VCP): initiator, modifier, and potential drug target for neurodegenerative diseases

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Chu, Siwei, Xie, Xinyi, Payan, Carla, Stochaj, Ursula·2023·Molecular Neurodegeneration
DOI 10.1186/s13024-023-00639-yPMC PMC10405438
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Abstract

The AAA+ATPase valosin containing protein (VCP) is essential for cell and organ homeostasis, especially in cells of the nervous system. As part of a large network, VCP collaborates with many cofactors to ensure proteostasis under normal, stress, and disease conditions. A large number of mutations have revealed the importance of VCP for human health. In particular, VCP facilitates the dismantling of protein aggregates and the removal of dysfunctional organelles. These are critical events to prevent malfunction of the brain and other parts of the nervous system. In line with this idea, VCP mutants are linked to the onset and progression of neurodegeneration and other diseases. The intricate molecular mechanisms that connect VCP mutations to distinct brain pathologies continue to be uncovered. Emerging evidence supports the model that VCP controls cellular functions on multiple levels and in a cell type specific fashion. Accordingly, VCP mutants derail cellular homeostasis through several mechanisms that can instigate disease. Our review focuses on the association between VCP malfunction and neurodegeneration. We discuss the latest insights in the field, emphasize open questions, and speculate on the potential of VCP as a drug target for some of the most devastating forms of neurodegeneration.

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  • SHIP1 is a membrane-associated lipid phosphatase that removes the 5' phosphate from PI(3,4,5)P3 to generate PI(3,4)P2, thereby negatively regulating PI3K/AKT signaling.

    “Src homology 2 (SH2) domain containing inositol polyphosphate 5-phosphatase 1 (SHIP1) is a membrane-associated lipid phosphatase that removes the 5’ phosphate from the second messenger PI(3,4,5)P3 to generate PI(3,4)P2, thereby negatively regulating PI3K/AKT signaling.”

    From INPP5D/SHIP1 regulates endo-lysosomal function and cargo-selective phagocytosis in human microglia (2025) · cites this paper as [1]

    The ‘VCP and lipid metabolism’ section discusses lipid droplets and cholesterol regulation, not SHIP1. Searches of our copy for SHIP1, the PI(3,4,5)P3-to-PI(3,4)P2 reaction, and PI3K/AKT signaling found no passage addressing the claim. The cited paper therefore does not verify it in our copy. (LLM, conf=0.77)

    Experimental — accuracy not yet measured

  • SHIP1 functions as a scaffolding protein through its additional domains, enabling interactions with proteins containing ITIMs/ITAMs, as well as SH2 and SH3 domains.

    “In addition to its phosphatase activity, SHIP1 functions as a scaffolding protein through its additional domains, enabling interactions with proteins containing immunoreceptor tyrosine inhibitory or activating motifs (ITIMs/ITAMs), as well as SH2 and SH3 domains1.”

    From INPP5D/SHIP1 regulates endo-lysosomal function and cargo-selective phagocytosis in human microglia (2025) · cites this paper as [1]

    The cited paper’s sections on VCP protein complexes and biochemical properties (page numbers unavailable) discuss VCP cofactors and ATPase activity, not SHIP1. Searches for SHIP1 scaffolding, ITIMs/ITAMs, and SH2/SH3 interactions found no passage addressing the claim. (LLM, conf=0.76)

    Experimental — accuracy not yet measured

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Authors
Chu, S., Xie, X., Payan, C., & Stochaj, U.
Affiliations
McGill University (CA)
Corresponding author
Stochaj, Ursula
ORCID
Venue
Molecular Neurodegeneration
Publisher
Springer Science and Business Media LLC
Published
2023-08-07
Volume
18
Issue
1
Pages
52-52
ISSN
1750-1326 (ISSN-L)
DOI
10.1186/s13024-023-00639-y
PMC
PMC10405438
OpenAlex
W4385620314
Cited by
58
References
18
Citations by year
2026: 19 · 2025: 26 · 2024: 13
FWCI
2.24
Type
journal article
Language
en
Open access
Licence
Locations
  • Molecular Neurodegeneration (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
Funders
  • Natural Sciences and Engineering Research Council of Canada
  • Fonds Québécois de la Recherche sur la Nature et les Technologies
  • Mitacs
Topics
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Heat shock proteins research
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Ubiquitin and proteasome pathways
Keywords
VCP, neurodegenerative diseases, proteostasis, protein aggregates, autophagy, VCP mutations, therapeutic target, cellular homeostasis
MeSH terms
Valosin Containing Protein, Proteostasis, Adenosine Triphosphatases, Humans, Mutation, Cell Cycle Proteins, Neurodegenerative Diseases

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