Evidence map›Paper›PMID 34437820›Full record

ReviewAnnual review of pathology2022

Cystic Fibrosis and the Cells of the Airway Epithelium: What Are Ionocytes and What Do They Do?

Viral S Shah, Raghu R Chivukula, Brian Lin, Avinash Waghray, Jayaraj Rajagopal

Open access · bronzeAbstract readReview
In one paragraph

Review in Annual review of pathology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers.

0numbers the graph read from it
0cells of the map it votes in
35citing papers in PubMed
5.1field-weighted citation impact, top 4% 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

35 citing papers in PubMed, 42 citations in OpenAlex.

  1. Refueling T cell memory in cystic fibrosis.American journal of respiratory cell and molecular biology · 2026
    Article
  2. Review
  3. Review
  4. Review
  5. Review
  6. Article
  7. Review
  8. Do pulmonary ionocytes absorb chloride or secrete chloride?American journal of physiology. Cell physiology · 2025
    Review
  9. Article
  10. Review
  11. Article
  12. Article
  13. Pulmonary Ionocytes Regulate Airway Surface Liquid pH in Primary Human Bronchial Epithelial Cells.American journal of respiratory and critical care medicine · 2024
    Article
  14. Article
  15. Review
  16. Article
  17. Review
  18. Review
  19. Article
  20. 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

5 authors at 4 institutions in 1 country.

Viral S ShahDepartment of Medicine, Division of Pulmonary and Critical Care Medicine, Massachusetts General Hospital, Boston, Massachusetts 02114, USA; email: vsshah@mgh.harvard.edu, rchivukula@mgh.harvard.edu, blin6@mgh.harvard.edu, awaghray@mgh.harvard.edu, jrajagopal@mgh.harvard.edu.
Raghu R ChivukulaDepartment of Medicine, Division of Pulmonary and Critical Care Medicine, Massachusetts General Hospital, Boston, Massachusetts 02114, USA; email: vsshah@mgh.harvard.edu, rchivukula@mgh.harvard.edu, blin6@mgh.harvard.edu, awaghray@mgh.harvard.edu, jrajagopal@mgh.harvard.edu.
Brian LinDepartment of Medicine, Division of Pulmonary and Critical Care Medicine, Massachusetts General Hospital, Boston, Massachusetts 02114, USA; email: vsshah@mgh.harvard.edu, rchivukula@mgh.harvard.edu, blin6@mgh.harvard.edu, awaghray@mgh.harvard.edu, jrajagopal@mgh.harvard.edu.
Avinash WaghrayDepartment of Medicine, Division of Pulmonary and Critical Care Medicine, Massachusetts General Hospital, Boston, Massachusetts 02114, USA; email: vsshah@mgh.harvard.edu, rchivukula@mgh.harvard.edu, blin6@mgh.harvard.edu, awaghray@mgh.harvard.edu, jrajagopal@mgh.harvard.edu.
Jayaraj RajagopalDepartment of Medicine, Division of Pulmonary and Critical Care Medicine, Massachusetts General Hospital, Boston, Massachusetts 02114, USA; email: vsshah@mgh.harvard.edu, rchivukula@mgh.harvard.edu, blin6@mgh.harvard.edu, awaghray@mgh.harvard.edu, jrajagopal@mgh.harvard.edu.
Massachusetts General Hospital · USHarvard Stem Cell Institute · USMassachusetts Institute of Technology · USWhitehead Institute for Biomedical Research · US

Funding

Research Training in Pulmonary Immunology and Allergy at MGHT32HL116275 · NHLBI · MASSACHUSETTS GENERAL HOSPITAL · PI David C Christiani, ANDREW D LUSTER · 2013 to 2026
$8.6M
Intercellular communication and cell regulation in airway epithelial ensembles in regeneration and diseaseR01HL118185 · NHLBI · MASSACHUSETTS GENERAL HOSPITAL · PI RAJAGOPAL, JAYARAJ · 2014 to 2021
$4.2M
Mechanisms governing the physiologic regulation of cell fate: Hypoxia-induced differentiation of neuroendocrine cells from stem cellsR01HL148351 · NHLBI · MASSACHUSETTS GENERAL HOSPITAL · PI RAJAGOPAL, JAYARAJ · 2020 to 2023
$2.4M
Live Imaging of Intact AirwayR01HL142559 · NHLBI · MASSACHUSETTS GENERAL HOSPITAL · PI LIN, CHARLES P., RAJAGOPAL, JAYARAJ · 2018 to 2021
$2.2M
Howard Hughes Medical InstituteNHLBI NIH HHS R01 HL118185NHLBI NIH HHS R01 HL142559NHLBI NIH HHS R01 HL148351NHLBI NIH HHS T32 HL116275
6 · The paper itself

Abstract

Cystic fibrosis (CF) is caused by defects in an anion channel, the cystic fibrosis transmembrane conductance regulator (CFTR). Recently, a new airway epithelial cell type has been discovered and dubbed the pulmonary ionocyte. Unexpectedly, these ionocytes express higher levels of CFTR than any other airway epithelial cell type. However, ionocytes are not the sole CFTR-expressing airway epithelial cells, and CF-associated disease genes are in fact expressed in multiple airway epithelial cell types. The experimental depletion of ionocytes perturbs epithelial physiology in the mouse trachea, but the role of these rare cells in the pathogenesis of human CF remains mysterious. Ionocytes have been described in diverse tissues(kidney and inner ear) and species (frog and fish). We draw on these prior studies to suggest potential roles of airway ionocytes in health and disease. A complete understanding of ionocytes in the mammalian airway will ultimately depend on cell type-specific genetic manipulation.

Indexed as

Cystic FibrosisAnimalsCystic Fibrosis Transmembrane Conductance RegulatorEpithelial CellsEpitheliumHumansMiceCystic Fibrosis Transmembrane Conductance RegulatorairwayCFTRcystic fibrosisepithelial cellionocytemitochondria-rich cell

Identifiers

PMID34437820
PMCPMC10837786
OpenAlexW3193344543

What OpenQuestion holds

Textmetadata
LicenceTDM
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.