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Article

Humanized neurofibroma model from induced pluripotent stem cells delineates tumor pathogenesis and developmental origins.

The Journal of clinical investigation - 4 Jan 2021

Mo Juan, Anastasaki Corina, Chen Zhiguo, Shipman Tracey, Papke Jason, Yin Kevin, Gutmann David H, Le Lu Q

Abstract excerpt

Neurofibromatosis type 1 (NF1) is a common tumor predisposition syndrome caused by NF1 gene mutation, in which affected patients develop Schwann cell lineage peripheral nerve sheath tumors (neurofibromas). To investigate human neurofibroma pathogenesis, we differentiated a series of isogenic, patient-specific NF1-mutant human induced pluripotent stem cells (hiPSCs) into Schwannian lineage cells (SLCs). We found...

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Identifiers

PMID: 33108355
DOI: 10.1172/JCI139807
PMCID: PMC7773354

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