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Cited by 36Immunodeficiency and Autoimmune DisordersT-cell and B-cell ImmunologyProtein Kinase Regulation and GTPase Signaling

The autoinhibitory C-terminal SH2 domain of phospholipase C–γ2 stabilizes B cell receptor signalosome assembly

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Wang, Jing, Sohn, Haewon, Sun, Guangping, Milner, Joshua D., Pierce, Susan K.·2014·Science Signaling
DOI 10.1126/scisignal.2005392PMC PMC4180509
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Abstract

The immune disorder PLAID is found to be the result of destabilization of B cell receptor complexes due to loss of an autoinhibitory domain in phospholipase C–γ2.

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  • In humans, seven cases of autoinflammation caused by PLCγ2 gain-of-function point mutations, summarized under the acronym APLAID, have been reported.

    “In humans, seven cases of autoinflammation caused by PLCγ2 gain-of-function point mutations, summarized under the acronym APLAID, have been reported (69).”

    From The role of PLCγ2 in immunological disorders, cancer, and neurodegeneration (2021) · cites this paper as [69]

    The Discussion (page not provided) describes APLAID patients with a gain-of-function PLC-γ2 point mutation, S707Y. It does not report seven cases; searches for that number and for APLAID case counts found no supporting passage in our copy. (LLM, conf=0.88)

    Experimental — accuracy not yet measured

  • Impaired cellular movement of the antigen-engaged BCR may be responsible, at least in part, for PLAID through reduction of specific antigen engagement by T and B cells required to activate and differentiate B cells allowing antibody secretion and memory formation.

    “The authors of this study concluded that the impaired cellular movement of the antigen-engaged BCR may be responsible, at least in part, for the PLAID syndrome through reduction of specific antigen engagement by T and B cells that are required to activate and differentiate B cells allowing antibody secretion and formation of B cell memory (69).”

    From The role of PLCγ2 in immunological disorders, cancer, and neurodegeneration (2021) · cites this paper as [69]

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References 50 · Cited by 36

Research works printed but not linked: 44

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Authors
Wang, J., Sohn, H., Sun, G., Milner, J. D., & Pierce, S. K.
Affiliations
  • National Institute of Allergy and Infectious Diseases (US)
  • National Institutes of Health (US)
Corresponding author
  • Wang, Jing
  • Sohn, Haewon
  • Pierce, Susan K.
ORCID
Venue
Science Signaling
Publisher
American Association for the Advancement of Science (AAAS)
Published
2014-09-16
Volume
7
Issue
343
Pages
ra89-ra89
ISSN
  • 1937-9145 (ISSN-L)
  • 1945-0877
DOI
10.1126/scisignal.2005392
PMC
PMC4180509
OpenAlex
W2314340678
Cited by
36
References
50
Citations by year
2026: 1 · 2025: 4 · 2024: 1 · 2023: 4 · 2022: 2 · 2021: 3 · 2020: 8 · 2019: 4 · 2018: 4 · 2017: 1 · 2016: 1 · 2015: 3
FWCI
0.76
Type
journal article
Language
en
Open access
Locations
  • Science Signaling (publishedVersion)
  • PubMed (publishedVersion)
  • Europe PMC (PubMed Central) (acceptedVersion)
  • PubMed Central (acceptedVersion)
Funders
  • National Institutes of Health
  • National Institute of Allergy and Infectious Diseases (ZIAAI001126)
Topics
  • Life Sciences › Immunology and Microbiology › Immunology › Immunodeficiency and Autoimmune Disorders
  • Life Sciences › Immunology and Microbiology › Immunology › T-cell and B-cell Immunology
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Protein Kinase Regulation and GTPase Signaling
Keywords
PLCγ2, autoinhibition, B cell receptor signaling, signalosome assembly, PLCG2 mutations, Syk, Bruton's tyrosine kinase, BLNK, Lyn kinase, calcium signaling
MeSH terms
Syk Kinase, Agammaglobulinaemia Tyrosine Kinase, Analysis of Variance, Animals, Calcium, Chlorocebus aethiops, Flow Cytometry, Humans, Immunologic Deficiency Syndromes, Lipid Bilayers, Microscopy, Fluorescence, Phosphorylation, Protein-Tyrosine Kinases, Receptors, Antigen, B-Cell, Signal Transduction, Microscopy, Confocal, src Homology Domains, COS Cells, Multiprotein Complexes, Intracellular Signaling Peptides and Proteins, Phospholipase C gamma

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