Abstract
Targeting oncogenic pathways holds promise for brain tumor treatment, but inhibition of Sonic Hedgehog (SHH) signaling has failed in SHH-driven medulloblastoma. Cellular diversity within tumors and reduced lineage commitment can undermine targeted therapy by increasing the probability of treatment-resistant populations. Using single-cell RNA-seq and lineage tracing, we analyzed cellular diversity in medulloblastomas in transgenic, medulloblastoma-prone mice, and responses to the SHH-pathway inhibitor vismodegib. In untreated tumors, we find expected stromal cells and tumor-derived cells showing either a spectrum of neural progenitor-differentiation states or glial and stem cell markers. Vismodegib reduces the proliferative population and increases differentiation. However, specific cell types in vismodegib-treated tumors remain proliferative, showing either persistent SHH-pathway activation or stem cell characteristics. Our data show that even in tumors with a single pathway-activating mutation, diverse mechanisms drive tumor growth. This diversity confers early resistance to targeted inhibitor therapy, demonstrating the need to target multiple pathways simultaneously.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
MeSH terms
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Anilides / pharmacology
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Anilides / therapeutic use
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Animals
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Cell Proliferation / drug effects
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Cell Proliferation / genetics
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Cerebellar Neoplasms / drug therapy
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Cerebellar Neoplasms / genetics*
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Cerebellar Neoplasms / pathology
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Cerebellum / cytology
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Cerebellum / pathology
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Drug Resistance, Neoplasm / genetics*
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Female
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Gain of Function Mutation
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Hedgehog Proteins / antagonists & inhibitors*
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Hedgehog Proteins / genetics
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Humans
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Male
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Medulloblastoma / drug therapy
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Medulloblastoma / genetics*
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Medulloblastoma / pathology
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Mice
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Mice, Transgenic
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Molecular Targeted Therapy / methods
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MyoD Protein / genetics
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Neoplastic Stem Cells / drug effects
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Pyridines / pharmacology
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Pyridines / therapeutic use
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RNA-Seq
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Signal Transduction / drug effects
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Signal Transduction / genetics*
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Single-Cell Analysis
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Smoothened Receptor / genetics
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Transcription Factor HES-1 / genetics
Substances
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Anilides
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Hedgehog Proteins
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Hes1 protein, mouse
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HhAntag691
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MyoD Protein
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MyoD1 myogenic differentiation protein
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Pyridines
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SHH protein, human
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Shh protein, mouse
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Smo protein, mouse
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Smoothened Receptor
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Transcription Factor HES-1
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HES1 protein, human