Table 2.
Summary of preclinical models of MB
| Classification | MB subgroup | MB incidence |
|---|---|---|
| Genetically engineered mouse models (GEMMs) | ||
| Ptc+/− | SHH | 14% |
| Ptc+/− p53−/− | SHH | 95% |
| Ptc+/− Ink4c+/− or −/− | SHH | 30% |
| Ptc+/− Kip1+/− or −/− | SHH | 60–70% |
| Ptc+/− Hic1−/− | SHH | ∼40% |
| Math1–Cre/Ptc C/C | SHH | 100% |
| Gfap–Cre/Ptc C/C | SHH | 100% |
| Sufu+/−/P53−/− | SHH | 58% |
| Hemizygous ND2–SmoA | SHH | 48% |
| Homozygous ND2–SmoA | SHH | 94% |
| Gfap–Cre/Rb loxp/loxp/Tp53 −/− or loxp/loxp | SHH | >84% |
| Lig4−/−/p53−/− | SHH | 100% |
| Nestin–Cre/Xrcc4 loxp/loxp/p53−/− | SHH | 87% |
| Nestin–Cre/Xrcc2 loxp/loxp/p53−/− | SHH | >90% |
| Nestin–Cre/Lig4 loxp/loxp/p53−/− | SHH | >90% |
| Nestin–Cre/Brca2 loxp/loxp/p53−/− | SHH | >90% |
| Parp1−/−/p53−/− | SHH | 49% |
| GTML | Classic or LCA | 75% |
| Blbp–Cre/Ctnnb1+/lox(ex3)/Tp53 flx/flx | WNT | 15% |
| Retroviral mouse model | ||
| SHH expressing retrovirus | SHH | 76% |
| Transplanted transduced cells or RCAS/tv-a system | ||
| Neural progenitors with RCAS (SHH + AKT) | SHH | 48% |
| Neural progenitors with RCAS (SHH + IGF2) | SHH | 39% |
| Neural progenitors with RCAS (SHH + MYC) | SHH? | 23% |
| Neural progenitors with RCAS (SHH + MYCN T50A) | SHH | 78% |
| p53 null neural progenitors with RCAS (MYC) | Group 3? | 50% |
| Neural progenitors with RCAS (MYC + Bcl2) | Group 3? | 18% |
| p53 null cerebellar progenitor cells transduced with MYC | Group 3? | 100% |
| Cerebellar stem cells expressing MYC and mutant p53 | Group 3? | 33% |
| Patient-derived xenograft models (PDXs) | ||
| Icb-1192 | WNT | N/A |
| Icb-1140 | WNT | N/A |
| Icb-1338 | SHH | N/A |
| Ic-984 | SHH | N/A |
| HD-MB03 | Group 3 | N/A |
| MB3W1 | Group 3 | 100% |
| Icb-1572 | Group 3 | N/A |
| Icb-1494 | Group 3 | N/A |
| Icb-1595 | Group 3 | N/A |
| Icb-1197 | Group 3 | N/A |
| Icb-1299 | Group 4 | N/A |
| Icb-1078 | Group 4 | N/A |
| Icb-1487 | Group 4 | N/A |
LCA: large cell anaplastic, N/A: not available.