TY - CHAP
T1 - Using genetically engineered mouse models to understand low-grade glioma development and growth in children
AU - Kaul, Aparna
AU - Hussain, Ibrahim
AU - Gutmann, David H.
PY - 2013
Y1 - 2013
N2 - Pilocytic astrocytoma (PA) is the most common brain tumor observed in children. These tumors can form sporadically in children with no underlying genetic disease or in 15-20% of children with neurofibromatosis type 1 (NF1), an inherited cancer predisposition syndrome. Though similar histologically, the genetic basis for PA formation in these two populations are distinct. In the general population, PAs likely arise in response to aberrant BRAF activation, whereas in the context of NF1, they result from bi-allelic inactivation of the NF1 tumor suppressor gene. Since accurate rodent models of sporadic PA are currently under development, Nf1 genetically engineered mouse models have served as tractable systems to study the role of aberrant intracellular signaling, nonneoplastic cells in the tumor microenvironment, and genomic modifiers on gliomagenesis. These small-animal models have also been used as platforms to discover next-generation targeted therapies and to evaluate the efficacy of these potential anticancer treatments prior to clinical trials for NF1-associated astrocytomas.
AB - Pilocytic astrocytoma (PA) is the most common brain tumor observed in children. These tumors can form sporadically in children with no underlying genetic disease or in 15-20% of children with neurofibromatosis type 1 (NF1), an inherited cancer predisposition syndrome. Though similar histologically, the genetic basis for PA formation in these two populations are distinct. In the general population, PAs likely arise in response to aberrant BRAF activation, whereas in the context of NF1, they result from bi-allelic inactivation of the NF1 tumor suppressor gene. Since accurate rodent models of sporadic PA are currently under development, Nf1 genetically engineered mouse models have served as tractable systems to study the role of aberrant intracellular signaling, nonneoplastic cells in the tumor microenvironment, and genomic modifiers on gliomagenesis. These small-animal models have also been used as platforms to discover next-generation targeted therapies and to evaluate the efficacy of these potential anticancer treatments prior to clinical trials for NF1-associated astrocytomas.
KW - Astrocytoma
KW - Brain tumor
KW - Genetically engineered mice
KW - Optic glioma
KW - Pilocytic astrocytoma
UR - http://www.scopus.com/inward/record.url?scp=84870543719&partnerID=8YFLogxK
U2 - 10.1007/7657_2011_29
DO - 10.1007/7657_2011_29
M3 - Chapter
AN - SCOPUS:84870543719
SN - 9781627032087
T3 - Neuromethods
SP - 203
EP - 215
BT - Animal Models of Brain Tumors
A2 - Murillo, Ricardo Martinez
A2 - Martinez, Alfredo
ER -