Medulloblastoma: mouse models and novel targeted therapies based on the Sonic hedgehog pathway.
Understanding molecular pathways, signaling cascades, and genetic alterations activated during tumorigenesis is essential for the development of targeted cancer treatments. In children, tumors of the central nervous system are thought to arise from progenitor cells that show considerable temporal and spatial heterogeneity in a developmental environment that is different from that of the adult. Investigating the molecular basis of pediatric tumors is critical because it is likely to generate novel treatments. Animal models have brought many important advances in this field. In this review the authors discuss the mouse models based on the Sonic hedgehog pathway, which have provided a better knowledge of the genetic and molecular alterations of medulloblastoma.
Piedimonte
Leandro R
LR
Division of Pediatric Neurosurgery, Department of Neurosurgery, New York University School of Medicine, New York, New York 10016, USA.
Wailes
Ian K
IK
Weiner
Howard L
HL
D000818:Animals; D002528:Cerebellar Neoplasms / Q000235:genetics* / Q000378:metabolism / Q000628:therapy; D004195:Disease Models, Animal; D018390:Gene Targeting / Q000379:methods*; D053823:Hedgehog Proteins; D008527:Medulloblastoma / Q000235:genetics* / Q000378:metabolism / Q000628:therapy; D051379:Mice; D015398:Signal Transduction / Q000235:genetics; D015534:Trans-Activators / Q000235:genetics* / Q000502:physiology
D053823:Hedgehog Proteins; C465570:Shh protein, mouse; D015534:Trans-Activators