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Model Characteristics - Model:
Nf1+/-GFAPCKO
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| Model Descriptor |
Nf1+/-GFAPCKO
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| Official Nomenclature |
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| Genotype |
Nf1+/-GFAPCKO
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| Species |
Mouse (Mus musculus)
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| Strain |
Not specified
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| Is This a Tool Strain? |
No
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Developmental Stage
(applies only to Zebrafish) |
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| Experimental Design |
We analyzed three genetically engineered mouse (GEM) models of low-grade glioma resulting from either inactivation of the neurofibromatosis-1 (Nf1) tumor suppressor gene (Nf1+/-supGFAP/supCKO and a href=http://cancermodels.nci.nih.gov/camod/ViewModelAction.do?unprotected_method=populateModelCharacteristics&aModelID=50057345 target="_blank" Nf1+/-supGFAP-m/supCKO/a) or constitutive activation of KRas (a href=http://cancermodels.nci.nih.gov/camod/ViewModelAction.do?unprotected_method=populateModelCharacteristics&aModelID=50057351 target="_blank" Nf1+/-supKRas/sup/a) in glial cells. Based on tumor proliferation, location, and penetrance, we selected this GEM model, Nf1+/-supGFAP/supCKO, for preclinical drug evaluation.brbr
The Nf1+/-supGFAP/supCKO mice were generated by successive breeding of NF1+/- mice with Nf1supflox/flox/sup mice and GFAP-Cre mice. 100% of the mice develop prechiasmatic and chiasmatic optic gliomas. The mice were randomized to either treatment or control groups, after verifying the presence of an optic glioma by manganese enhanced MRI at 10-12 weeks. The three treatment groups were temozolomide treatment (trial 1), rapamycin treatment (trial 2) or temozolomide and rapamycin treatment (trial 3).
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| Phenotype |
The optic gliomas in the Nf1+/-GFAPCKO mice showed increased cell death, decreased cell proliferation, and reduced tumor volume when treated with agents that are used in conventional chemotherapy in children with optic gliomas. Of the three models evaluated, this one displayed characteristics most closely resembling human pediatric low-grade gliomas.
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| Website for add. info |
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| Breeding Notes |
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| Sex Distribution of the Phenotype |
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| Submitted by |
caMOD, Curator
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| Principal Investigator / Lab |
Gutmann, David
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| Comment |
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| Model Availability: This model is available from |
| Strain |
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