Evidence map›Paper›PMID 32730934›Full record

ReviewBone2020

Fibrodysplasia ossificans progressiva (FOP): A disorder of osteochondrogenesis.

Frederick S Kaplan, Mona Al Mukaddam, Alexandra Stanley, O Will Towler, Eileen M Shore

Open access · greenAbstract readReview
In one paragraph

Review in Bone, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.

0numbers the graph read from it
0cells of the map it votes in
28citing papers in PubMed
4.9field-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

28 citing papers in PubMed, 43 citations in OpenAlex.

  1. Article
  2. Article
  3. Article
  4. Article
  5. Article
  6. Article
  7. Review
  8. Rare genetic skeletal disorders: Evolving terminology, therapies, education and advocacy.Journal of the Pediatric Orthopaedic Society of North America · 2024
    Article
  9. Article
  10. Article
  11. Article
  12. Article
  13. Article
  14. BMP signaling in cancer stemness and differentiation.Cell regeneration (London, England) · 2023
    Review
  15. Review
  16. Fibrodysplasia ossificans progressiva.Clinical case reports · 2023
    Article
  17. Article
  18. Review
  19. Article
  20. Journal of dental research · 2022
    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 1 institution in 1 country.

Frederick S KaplanDepartment of Orthopaedic Surgery, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA 19104, USA; Department of Medicine, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA 19104, USA; The Center for Research in FOP & Related Disorders, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address: Frederick.Kaplan@pennmedicine.upenn.edu.
Mona Al MukaddamDepartment of Orthopaedic Surgery, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA 19104, USA; Department of Medicine, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA 19104, USA; The Center for Research in FOP & Related Disorders, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address: Mona.AlMukaddam@pennmedicine.upenn.edu.
Alexandra StanleyDepartment of Orthopaedic Surgery, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA 19104, USA; The Center for Research in FOP & Related Disorders, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address: astanley@health.ucsd.edu.
O Will TowlerDepartment of Orthopaedic Surgery, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA 19104, USA; The Center for Research in FOP & Related Disorders, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address: otowler@pennmedicine.upenn.edu.
Eileen M ShoreDepartment of Orthopaedic Surgery, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA 19104, USA; Department of Genetics, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA 19104, USA; The Center for Research in FOP & Related Disorders, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address: shore@pennmedicine.upenn.edu.
University of Pennsylvania · US

Funding

Overall: Resource-based Center for Musculoskeletal Disorders Research (Overall Application)P30AR069619 · NIAMS · UNIVERSITY OF PENNSYLVANIA · PI LOUIS J SOSLOWSKY · 2016 to 2026
$9.1M
Mechanobiology of Progenitor Cells in Heterotopic OssificationR01AR071399 · NIAMS · UNIVERSITY OF PENNSYLVANIA · PI MAUCK, ROBERT L, SHORE, EILEEN M · 2018 to 2022
$1.6M
NIAMS NIH HHS P30 AR069619NIAMS NIH HHS R01 AR071399
6 · The paper itself

Abstract

Fibrodysplasia ossificans progressiva (FOP) is an ultra-rare genetic disorder of extraskeletal bone formation, but could appropriately be viewed as a seminal disorder of osteochondrogenesis. Many, if not most, of the musculoskeletal features of FOP are related to dysregulated chondrogenesis including abnormal articular cartilage formation, abnormal diarthrodial joint specification, growth plate dysplasia, osteochondroma formation, heterotopic endochondral ossification (HEO), and precocious arthropathy. In FOP, causative activating mutations of Activin receptor A type I (ACVR1), a bone morphogenetic protein (BMP) type I receptor, are responsible for the osteochondrodysplasia that impacts developmental phenotypes as well as postnatal features of this illustrative disorder. Here, we highlight the myriad developmental and postnatal effects on osteochondrogenesis that emanate directly from mutant ACVR1 and dysregulated bone morphogenetic protein (BMP) signaling in FOP.

Indexed as

Myositis OssificansOssification, HeterotopicActivin Receptors, Type IBone Morphogenetic ProteinsChondrogenesisHumansActivin Receptors, Type IBone Morphogenetic ProteinsACVR1ArthropathyBone morphogenetic protein signaling pathwayDegenerative joint diseaseFibrodysplasia ossificans progressivaGrowth plateHeterotopic ossificationOsteochondrodysplasiaOsteochondrogenesisOsteochondroma

Identifiers

PMID32730934
PMCPMC7502483
OpenAlexW3045572912

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.