TY - JOUR
T1 - Advanced pediatric diffuse pontine glioma murine models pave the way towards precision medicine
AU - Chen, Zirong
AU - Peng, Peng
AU - Zhang, Xiaolin
AU - Mania-Farnell, Barbara
AU - Xi, Guifa
AU - Wan, Feng
N1 - Funding Information:
This work was funded by National Natural Science Foundation of China, grant number 82072795.
Publisher Copyright:
© 2021 by the authors. Licensee MDPI, Basel, Switzerland.
PY - 2021/3/1
Y1 - 2021/3/1
N2 - Diffuse intrinsic pontine gliomas (DIPGs) account for ~15% of pediatric brain tumors, which invariably present with poor survival regardless of treatment mode. Several seminal studies have revealed that 80% of DIPGs harbor H3K27M mutation coded by HIST1H3B, HIST1H3C and H3F3A genes. The H3K27M mutation has broad effects on gene expression and is considered a tumor driver. Determination of the effects of H3K27M on posttranslational histone modifications and gene regulations in DIPG is critical for identifying effective therapeutic targets. Advanced animal models play critical roles in translating these cutting-edge findings into clinical trial development. Here, we review current molecular research progress associated with DIPG. We also summarize DIPG animal models, highlighting novel genomic engineered mouse models (GEMMs) and innovative humanized DIPG mouse models. These models will pave the way towards personalized precision medicine for the treatment of DIPGs.
AB - Diffuse intrinsic pontine gliomas (DIPGs) account for ~15% of pediatric brain tumors, which invariably present with poor survival regardless of treatment mode. Several seminal studies have revealed that 80% of DIPGs harbor H3K27M mutation coded by HIST1H3B, HIST1H3C and H3F3A genes. The H3K27M mutation has broad effects on gene expression and is considered a tumor driver. Determination of the effects of H3K27M on posttranslational histone modifications and gene regulations in DIPG is critical for identifying effective therapeutic targets. Advanced animal models play critical roles in translating these cutting-edge findings into clinical trial development. Here, we review current molecular research progress associated with DIPG. We also summarize DIPG animal models, highlighting novel genomic engineered mouse models (GEMMs) and innovative humanized DIPG mouse models. These models will pave the way towards personalized precision medicine for the treatment of DIPGs.
KW - Diffuse intrinsic pontine glioma
KW - Genetically engineered mouse model
KW - Humanized mouse model
KW - Molecular biology
KW - Patient derived xenografts
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U2 - 10.3390/cancers13051114
DO - 10.3390/cancers13051114
M3 - Review article
C2 - 33807733
AN - SCOPUS:85102004686
VL - 13
SP - 1
EP - 17
JO - Cancers
JF - Cancers
SN - 2072-6694
IS - 5
M1 - 1114
ER -
