Evidence map›Paper›PMID 29386111›Full record

ArticleCell reports2018

Modeling Congenital Adrenal Hyperplasia and Testing Interventions for Adrenal Insufficiency Using Donor-Specific Reprogrammed Cells.

Gerard Ruiz-Babot, Mariya Balyura, Irene Hadjidemetriou, Sharon J Ajodha, David R Taylor, Lea Ghataore, Norman F Taylor, Undine Schubert, Christian G Ziegler, Helen L Storr and 9 more

Open access · goldAbstract read
In one paragraph

Article in Cell reports, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
35citing papers in PubMed, 1 pooled it
3.1field-weighted citation impact, top 8% 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, 1 synthesis or guideline pooled it, 61 citations in OpenAlex.

  1. Guideline
  2. Trial
  3. [Congenital adrenal hyperplasia].Problemy endokrinologii · 2026
    Review
  4. Review
  5. Article
  6. Review
  7. Review
  8. Article
  9. Article
  10. Article
  11. Review
  12. Models of Congenital Adrenal Hyperplasia for Gene Therapies Testing.International journal of molecular sciences · 2023
    Review
  13. Review
  14. Development and function of the fetal adrenal.Reviews in endocrine & metabolic disorders · 2023
    Review
  15. Review
  16. Novel treatments for congenital adrenal hyperplasia.Reviews in endocrine & metabolic disorders · 2022
    Review
  17. Review
  18. Article
  19. Article
  20. Review
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

19 authors at 8 institutions in 2 countries.

Gerard Ruiz-BabotCentre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, EC1M 6BQ London, UK.
Mariya BalyuraUniversity Hospital Carl Gustav Carus, Department of Medicine III, Technische Universität Dresden, 01307 Dresden, Germany.
Irene HadjidemetriouCentre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, EC1M 6BQ London, UK.
Sharon J AjodhaCentre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, EC1M 6BQ London, UK.
David R TaylorDepartment of Clinical Biochemistry, King's College Hospital NHS Foundation Trust, Denmark Hill, SE5 9RS London, UK.
Lea GhataoreDepartment of Clinical Biochemistry, King's College Hospital NHS Foundation Trust, Denmark Hill, SE5 9RS London, UK.
Norman F TaylorDepartment of Clinical Biochemistry, King's College Hospital NHS Foundation Trust, Denmark Hill, SE5 9RS London, UK.
Undine SchubertUniversity Hospital Carl Gustav Carus, Department of Medicine III, Technische Universität Dresden, 01307 Dresden, Germany.
Christian G ZieglerUniversity Hospital Carl Gustav Carus, Department of Medicine III, Technische Universität Dresden, 01307 Dresden, Germany.
Helen L StorrCentre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, EC1M 6BQ London, UK.
Maralyn R DruceCentre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, EC1M 6BQ London, UK.
Evelien F GeversCentre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, EC1M 6BQ London, UK.
William M DrakeCentre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, EC1M 6BQ London, UK.
Umasuthan SrirangalingamDepartment of Endocrinology, University College London Hospitals, NW1 2PG London, UK.
Gerard S ConwayDepartment of Endocrinology, University College London Hospitals, NW1 2PG London, UK.
Peter J KingCentre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, EC1M 6BQ London, UK.
Louise A MetherellCentre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, EC1M 6BQ London, UK.
Stefan R BornsteinUniversity Hospital Carl Gustav Carus, Department of Medicine III, Technische Universität Dresden, 01307 Dresden, Germany; Paul Langerhans Institute Dresden of Helmholtz Centre Munich at University Clinic Carl Gustav Carus of TU Dresden Faculty of Medicine, Technische Universität Dresden, DZD-German Centre for Diabetes Research, 01307 Dresden, Germany; Center for Regenerative Therapies, Technische Universität Dresden, 01307 Dresden, Germany; Diabetes and Nutritional Sciences Division, King's College London, WC2R 2LS London, UK.
Leonardo GuastiCentre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, EC1M 6BQ London, UK. Electronic address: l.guasti@qmul.ac.uk.
William Harvey Research Institute · GBKing's College Hospital NHS Foundation Trust · GBQueen Mary University of London · GBTU Dresden · DEDeutsches Diabetes-Zentrum e.V. · DEUniversity College Hospital · GBUniversity College London · GBUniversity Hospital Carl Gustav Carus · DE

Funding

Biotechnology and Biological Sciences Research Council BB/L002671/1Medical Research Council G0801265Medical Research Council MR/K020455/1
6 · The paper itself

Abstract

Adrenal insufficiency is managed by hormone replacement therapy, which is far from optimal; the ability to generate functional steroidogenic cells would offer a unique opportunity for a curative approach to restoring the complex feedback regulation of the hypothalamic-pituitary-adrenal axis. Here, we generated human induced steroidogenic cells (hiSCs) from fibroblasts, blood-, and urine-derived cells through forced expression of steroidogenic factor-1 and activation of the PKA and LHRH pathways. hiSCs had ultrastructural features resembling steroid-secreting cells, expressed steroidogenic enzymes, and secreted steroid hormones in response to stimuli. hiSCs were viable when transplanted into the mouse kidney capsule and intra-adrenal. Importantly, the hypocortisolism of hiSCs derived from patients with adrenal insufficiency due to congenital adrenal hyperplasia was rescued by expressing the wild-type version of the defective disease-causing enzymes. Our study provides an effective tool with many potential applications for studying adrenal pathobiology in a personalized manner and opens venues for the development of precision therapies.

Indexed as

Adrenal Hyperplasia, CongenitalInduced Pluripotent Stem CellsAdrenal Cortex HormonesAdrenal InsufficiencyCells, CulturedCellular Reprogramming TechniquesHumansModels, BiologicalAdrenal Cortex Hormonesadrenal cortexadrenal insufficiencycongenital adrenal hyperplasiadisease modelingNR5A1reprogrammingsteroidogenic cellssteroidogenic factor 1transplantationurine-derived stem cells

Identifiers

PMID29386111
PMCPMC5809617
OpenAlexW2785960912

What OpenQuestion holds

Textmetadata
LicenceCC BY
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.