ArticleThe Journal of clinical investigation1998
Increased calvaria cell differentiation and bone matrix formation induced by fibroblast growth factor receptor 2 mutations in Apert syndrome.
Article in The Journal of clinical investigation, 1998. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers.
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.
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.
Who cites it
39 citing papers in PubMed, 615 citations in OpenAlex.
- Decreased proliferation and altered differentiation in osteoblasts from genetically and clinically distinct craniosynostotic disorders.The American journal of pathology · 1999Trial
- Combining Mendelian Randomization and Experimental Validation to Reveal the Causal Relationship Between Hallux Valgus and Serum Metabolites and to Identify Their Therapeutic Targets and Relevant Components.Food science & nutrition · 2026Article
- Excessive osteoclast activation by osteoblast paracrine factor RANKL is a major cause of the abnormal long bone phenotype in Apert syndrome model mice.Journal of cellular physiology · 2022Article
- Erf Affects Commitment and Differentiation of Osteoprogenitor Cells in Cranial Sutures via the Retinoic Acid Pathway.Molecular and cellular biology · 2021Article
- Cranial Neural Crest Cells and Their Role in the Pathogenesis of Craniofacial Anomalies and Coronal Craniosynostosis.Journal of developmental biology · 2020Review
- Cell Mechanics of Craniosynostosis.ACS biomaterials science & engineering · 2017Article
- BMP9 induces osteogenesis and adipogenesis in the immortalized human cranial suture progenitors from the patent sutures of craniosynostosis patients.Journal of cellular and molecular medicine · 2017Article
- Fibroblast Growth Factor Receptor 2 (International journal of biological sciences · 2017Review
- Activation of the IGF1 pathway mediates changes in cellular contractility and motility in single-suture craniosynostosis.Journal of cell science · 2016Article
- A Genetic-Pathophysiological Framework for Craniosynostosis.American journal of human genetics · 2015Review
- Fibroblast growth factor signaling in skeletal development and disease.Genes & development · 2015Review
- Osteoblast dysfunctions in bone diseases: from cellular and molecular mechanisms to therapeutic strategies.Cellular and molecular life sciences : CMLS · 2015Review
- Inhibited Wnt signaling causes age-dependent abnormalities in the bone matrix mineralization in the Apert syndrome FGFR2(S252W/+) mice.PloS one · 2015Article
- Characterization of distinct classes of differential gene expression in osteoblast cultures from non-syndromic craniosynostosis bone.Journal of genomics · 2014Article
- Therapeutic effect of nanogel-based delivery of soluble FGFR2 with S252W mutation on craniosynostosis.PloS one · 2014Article
- The molecular and cellular basis of Apert syndrome.Intractable & rare diseases research · 2013Review
- Further analysis of the Crouzon mouse: effects of the FGFR2(C342Y) mutation are cranial bone-dependent.Calcified tissue international · 2013Article
- Postnatal brain and skull growth in an Apert syndrome mouse model.American journal of medical genetics. Part A · 2013Article
- E3 ubiquitin ligase-mediated regulation of bone formation and tumorigenesis.Cell death & disease · 2013Review
- Craniosynostosis-associated Fgfr2(C342Y) mutant bone marrow stromal cells exhibit cell autonomous abnormalities in osteoblast differentiation and bone formation.BioMed research international · 2013Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
8 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Apert syndrome, associated with fibroblast growth factor receptor (FGFR) 2 mutations, is characterized by premature fusion of cranial sutures. We analyzed proliferation and differentiation of calvaria cells derived from Apert infants and fetuses with FGFR-2 mutations. Histological analysis revealed premature ossification, increased extent of subperiosteal bone formation, and alkaline phosphatase- positive preosteoblastic cells in Apert fetal calvaria compared with age-matched controls. Preosteoblastic calvaria cells isolated from Apert infants and fetuses showed normal cell growth in basal conditions or in response to exogenous FGF-2. In contrast, the number of alkaline phosphatase- positive calvaria cells was fourfold higher than normal in mutant fetal calvaria cells with the most frequent Apert FGFR-2 mutation (Ser252Trp), suggesting increased maturation rate of cells in the osteoblastic lineage. Biochemical and Northern blot analyses also showed that the expression of alkaline phosphatase and type 1 collagen were 2-10-fold greater than normal in mutant fetal calvaria cells. The in vitro production of mineralized matrix formed by immortalized mutant fetal calvaria cells cultured in aggregates was also increased markedly compared with control immortalized fetal calvaria cells. The results show that Apert FGFR-2 mutations lead to an increase in the number of precursor cells that enter the osteogenic pathway, leading ultimately to increased subperiosteal bone matrix formation and premature calvaria ossification during fetal development, which establishes a connection between the altered genotype and cellular phenotype in Apert syndromic craniosynostosis.
Indexed as
Identifiers
9502772PMC508685W2148917087What OpenQuestion holds
Registered trials
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.