Evidence map›Paper›PMID 19513754›Full record

ReviewCell and tissue research2010

Biogenesis and function of fibrillin assemblies.

Francesco Ramirez, Lynn Y Sakai

Open access · greenAbstract readReview
In one paragraph

Review in Cell and tissue research, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 112 papers.

0numbers the graph read from it
0cells of the map it votes in
112citing papers in PubMed
10.0field-weighted citation impact, top 2% 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

112 citing papers in PubMed, 205 citations in OpenAlex.

  1. Article
  2. Article
  3. Abdominal aortic aneurysm progression: A review of preclinical and clinical data.Clinical research in cardiology : official journal of the German Cardiac Society · 2026
    Review
  4. Article
  5. Low-Dose HGlobal challenges (Hoboken, NJ) · 2026
    Article
  6. Article
  7. Article
  8. Article
  9. Review
  10. Article
  11. Article
  12. Article
  13. Article
  14. Article
  15. In Search of Mouse Models for Exfoliation Syndrome.American journal of ophthalmology · 2024
    Article
  16. Article
  17. Article
  18. Article
  19. Article
  20. Article

52 more citing papers are in PubMed but not listed here.

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

2 authors at 2 institutions in 1 country.

Francesco RamirezDepartment of Pharmacology and Systems Therapeutics, Mount Sinai School of Medicine, New York, NY, USA. francesco.ramirez@mssm.edu
Lynn Y Sakai
Icahn School of Medicine at Mount Sinai · USShriners Hospitals for Children - Portland · US

Funding

PROJECT 4: Exploration of Therapeutic Strategies in MarP01AR049698 · NIAMS · UNIV OF MED/DENT NJ-R W JOHNSON MED SCH · PI RAMIREZ, FRANCESCO B · 2004 to 2014
$13.9M
NOVEL MICROFIBRILLAR COMPONENTR01AR042044 · NIAMS · UNIV OF MED/DENT NJ-R W JOHNSON MED SCH · PI RAMIREZ, FRANCESCO B · 1993 to 2009
$3.0M
NIAMS NIH HHS AR-049698NIAMS NIH HHS AR-42044NIAMS NIH HHS P01 AR049698NIAMS NIH HHS R01 AR042044
6 · The paper itself

Abstract

Fibrillin-1 and fibrillin-2 are large cysteine-rich glycoproteins that serve two key physiological functions: as supporting structures that impart tissue integrity and as regulators of signaling events that instruct cell performance. The structural role of fibrillins is exerted through the temporal and hierarchical assembly of microfibrils and elastic fibers, whereas the instructive role reflects the ability of fibrillins to sequester transforming growth factor beta (TGFbeta) and bone morphogenetic protein (BMP) complexes in the extracellular matrix. Characterization of fibrillin mutations in human patients and in genetically engineered mice has demonstrated that perturbation of either function manifests in disease. More generally, these studies have indicated that fibrillins are integral components of a broader biological network of extracellular, cell surface, and signaling molecules that orchestrate morphogenetic and homeostatic programs in multiple organ systems. They have also suggested that the relative composition of fibrillin-rich microfibrils imparts contextual specificity to TGFbeta and BMP signaling by concentrating the ligands locally so as to regulate cell differentiation within a spatial context during organ formation (positive regulation) and by restricting their bioavailability so as to modulate cell performance in a timely fashion during tissue remodeling/repair (negative regulation). Correlative evidence suggests functional coupling of the cell-directed assembly of microfibrils and targeting of TGFbeta and BMP complexes to fibrillins. Hence, the emerging view is that fibrillin-rich microfibrils are molecular integrators of structural and instructive signals, with TGFbeta and BMPs as the nodal points that convert extracellular inputs into discrete and context-dependent cellular responses.

Indexed as

Signal TransductionAdipokinesAnimalsAnimals, Genetically ModifiedElastic TissueExtracellular MatrixFibrillin-1Fibrillin-2FibrillinsHumansMatrix Metalloproteinases, SecretedMiceMicrofilament ProteinsMutationOrganogenesisTransforming Growth Factor betaAdipokinesFBN1 protein, humanFbn1 protein, mouseFBN2 protein, humanFbn2 protein, mouseFibrillin-1Fibrillin-2FibrillinsMatrix Metalloproteinases, SecretedMicrofilament ProteinsTransforming Growth Factor beta

Identifiers

PMID19513754
PMCPMC2819175
OpenAlexW2105892139

What OpenQuestion holds

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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.