Evidence map›Paper›PMID 30911953›Full record

ReviewJournal of clinical immunology2019

Immune Dysregulation and Disease Pathogenesis due to Activating Mutations in PIK3CD-the Goldilocks' Effect.

Stuart G Tangye, Julia Bier, Anthony Lau, Tina Nguyen, Gulbu Uzel, Elissa K Deenick

Abstract readReview
PubMed Publisher
In one paragraph

Review in Journal of clinical immunology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.

0numbers the graph read from it
0cells of the map it votes in
26citing papers in PubMed
1.9field-weighted citation impact, top 13% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

26 citing papers in PubMed, 37 citations in OpenAlex.

  1. Trial
  2. Review
  3. Article
  4. Article
  5. Article
  6. Article
  7. Article
  8. The Importance of Phosphoinositide 3-Kinase in Neuroinflammation.International journal of molecular sciences · 2024
    Review
  9. Article
  10. Article
  11. Review
  12. Review
  13. PI3K Isoforms in B Cells.Current topics in microbiology and immunology · 2022
    Article
  14. Article
  15. PI3K inhibitors are finally coming of age.Nature reviews. Drug discovery · 2021
    Review
  16. Review
  17. Article
  18. Review
  19. PI3K in T Cell Adhesion and Trafficking.Frontiers in immunology · 2021
    Review
  20. Case Report: ActivatingFrontiers in immunology · 2021
    Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

6 authors at 4 institutions in 2 countries.

Stuart G TangyeImmunology, Garvan Institute of Medical Research, 384 Victoria St, Darlinghurst, NSW, 2010, Australia. s.tangye@garvan.org.au.
Julia BierImmunology, Garvan Institute of Medical Research, 384 Victoria St, Darlinghurst, NSW, 2010, Australia.
Anthony LauImmunology, Garvan Institute of Medical Research, 384 Victoria St, Darlinghurst, NSW, 2010, Australia.
Tina NguyenImmunology, Garvan Institute of Medical Research, 384 Victoria St, Darlinghurst, NSW, 2010, Australia.
Gulbu UzelLaboratory of Clinical Immunology and Microbiology, National Institutes of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
Elissa K DeenickImmunology, Garvan Institute of Medical Research, 384 Victoria St, Darlinghurst, NSW, 2010, Australia.
Garvan Institute of Medical Research · AUSt Vincent's Clinic · AUNational Institute of Allergy and Infectious Diseases · USUNSW Sydney · AU

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

"This porridge is too hot!" she exclaimed. So, she tasted the porridge from the second bowl. "This porridge is too cold," she said. So, she tasted the last bowl of porridge. "Ahhh, this porridge is just right," she said happily and she ate it all up. While this describes the adventures of Goldilocks in the classic fairytale "The Story of Goldilocks and the Three Bears," it is an ideal analogy for the need for balanced signaling mediated by phosphatidylinositol-3-kinase (PI3K), a key signaling hub in immune cells. Either too little or too much PI3K activity is deleterious, even pathogenic-it needs to be "just right"! This has been elegantly demonstrated by the identification of inborn errors of immunity in key components of the PI3K pathway, and the impact of these mutations on immune regulation. Detailed analyses of patients with germline activating mutations in PIK3CD, as well as the parallel generation of novel murine models of this disease, have shed substantial light on the role of PI3K in lymphocyte development and differentiation, and mechanisms of disease pathogenesis resulting not only from PIK3CD mutations but genetic lesions in other components of the PI3K pathway. Furthermore, by being able to pharmacologically target PI3K, these monogenic conditions have provided opportunities for the implementation of precision medicine as a therapy, as well as to gain further insight into the consequences of modulating the PI3K pathway in clinical settings.

Indexed as

AnimalsB-LymphocytesClass I Phosphatidylinositol 3-KinasesGain of Function MutationHumansPrimary Immunodeficiency DiseasesSignal TransductionT-LymphocytesClass I Phosphatidylinositol 3-Kinasesactivated PI3Kinase delta syndromecombined immunodeficiencyPI3 kinase

Identifiers

PMID30911953
OpenAlexW2927444267

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.