Cited by 150Sarcoma Diagnosis and TreatmentBone Tumor Diagnosis and TreatmentsPluripotent Stem Cells Research

Osteosarcoma: Molecular Pathogenesis and iPSC Modeling

Open access
Lin, Yu-Hsuan, Jewell, Brittany E., Gingold, Julian, Lu, Linchao, Zhao, Ruiying, Wang, Lisa L., Lee, Dung-Fang·2017·Trends in Molecular Medicine

Abstract

Rare hereditary disorders provide unequivocal evidence of the importance of genes in human disease pathogenesis. Familial syndromes that predispose to osteosarcomagenesis are invaluable in understanding the underlying genetics of this malignancy. Recently, patient-derived induced pluripotent stem cells (iPSCs) have been successfully utilized to model Li-Fraumeni syndrome (LFS)-associated bone malignancy, demonstrating that iPSCs can serve as an in vitro disease model to elucidate osteosarcoma etiology. We provide here an overview of osteosarcoma predisposition syndromes and review recently established iPSC disease models for these familial syndromes. Merging molecular information gathered from these models with the current knowledge of osteosarcoma biology will help us to gain a deeper understanding of the pathological mechanisms underlying osteosarcomagenesis and will potentially aid in the development of future patient therapies.

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Crossref · 10.1016/j.molmed.2017.06.004

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  • Introduction of TP53 mutations into partially differentiated osteogenic stem cells generated osteosarcoma-like cells in vitro.

    “Introduction of TP53 mutations into partially differentiated osteogenic stem cells generated osteosarcoma-like cells in vitro 31 .”

    From Osteosarcoma (2022) · cites this paper as [31]

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Authors
Lin, Y.-H., Jewell, B. E., Gingold, J., Lu, L., Zhao, R., Wang, L. L., & Lee, D.-F.
Affiliations
  • The University of Texas Health Science Center at Houston (US)
  • The University of Texas MD Anderson Cancer Center (US)
  • Cleveland Clinic (US)
  • Baylor College of Medicine (US)
Corresponding author
Lee, Dung-Fang
Venue
Trends in Molecular Medicine
Publisher
Elsevier BV
Published
2017-08
Volume
23
Issue
8
Pages
737-755
ISSN
1471-4914 (ISSN-L)
OpenAlex
W2737148036
Cited by
150
References
170
Citations by year
2026: 4 · 2025: 9 · 2024: 9 · 2023: 18 · 2022: 19 · 2021: 34 · 2020: 28 · 2019: 11 · 2018: 16 · 2017: 2
FWCI
2.85
Type
journal article
Language
en
Open access
Locations
  • Trends in Molecular Medicine (publishedVersion)
  • PubMed (publishedVersion)
  • Europe PMC (PubMed Central) (acceptedVersion)
  • PubMed Central (acceptedVersion)
Funders
  • National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) (AR059063)
  • Baylor College of Medicine (BCM) Intellectual and Developmental Disabilities Research Center (IDDRC) (P30HD024064)
Topics
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Sarcoma Diagnosis and Treatment
  • Health Sciences › Medicine › Rheumatology › Bone Tumor Diagnosis and Treatments
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Pluripotent Stem Cells Research
Keywords
osteosarcoma, Li-Fraumeni syndrome, hereditary cancer, induced pluripotent stem cells, iPSC disease models, TP53, in vitro disease model
MeSH terms
Animals, Bone Neoplasms, Humans, Models, Biological, Osteosarcoma, Li-Fraumeni Syndrome, Induced Pluripotent Stem Cells

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