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Cited by 224

The DOI matches its Crossref record; 7 of the 7 references it cites were found in a registry or an index.

Modeling Familial Cancer with Induced Pluripotent Stem Cells

Open access
Lee, Dung-Fang, Su, Jie, Kim, Huen Suk, Chang, Betty, Papatsenko, Dmitri, Zhao, Ruiying, Yuan, Ye, Gingold, Julian, Xia, Weiya, Darr, Henia, Mirzayans, Razmik, Hung, Mien-Chie, Schaniel, Christoph, Lemischka, Ihor R.·2015·Cell

Abstract

SUMMARY In vitro modeling of human disease has recently become feasible with induced pluripotent stem cell (iPSC) technology. Here, we established patient-derived iPSCs from a Li-Fraumeni Syndrome (LFS) family and investigated the role of mutant p53 in the development of osteosarcoma (OS). LFS iPSC-derived osteoblasts (OBs) recapitulated OS features including defective osteoblastic differentiation as well as tumorigenic ability. Systematic analyses revealed that the expression of genes enriched in LFS-derived OBs strongly correlated with decreased time to tumor recurrence and poor patient survival. Furthermore, LFS OBs exhibited impaired upregulation of the imprinted gene H19 during osteogenesis. Restoration of H19 expression in LFS OBs facilitated osteoblastic differentiation and repressed tumorigenic potential. By integrating human imprinted gene network (IGN) into functional genomic analyses, we found that H19 mediates suppression of LFS-associated OS through the IGN component DECORIN (DCN). In summary, these findings demonstrate the feasibility of studying inherited human cancer syndromes with iPSCs.

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Metadata

Authors
Lee, D.-F., Su, J., Kim, H. S., Chang, B., Papatsenko, D., Zhao, R., Yuan, Y., Gingold, J., Xia, W., Darr, H., Mirzayans, R., Hung, M.-C., Schaniel, C., & Lemischka, I. R.
Venue
Cell
Publisher
Elsevier BV
Published
2015-04
Volume
161
Issue
2
Pages
240-254
ISSN
0092-8674 (ISSN-L)
OpenAlex
W2121188788
Cited by
224
References
7
FWCI
10.29
Type
journal article
Language
en
Open access
bronze

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CoChat. (2026). Citation check: Modeling Familial Cancer with Induced Pluripotent Stem Cells. https://app.cochat.ai/papers/10.1016/j.cell.2015.02.045
The paper
Lee, D.-F., Su, J., Kim, H. S., Chang, B., Papatsenko, D., Zhao, R., Yuan, Y., Gingold, J., Xia, W., Darr, H., Mirzayans, R., Hung, M.-C., Schaniel, C., & Lemischka, I. R. (2015). Modeling Familial Cancer with Induced Pluripotent Stem Cells. Cell. https://doi.org/10.1016/j.cell.2015.02.045

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

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