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PMID: 11718263 Published · ppublish English Journal Article Review

A mouse model for medulloblastoma and basal cell nevus syndrome.

Journal of neuro-oncology ·Vol. 53 ·No. 3 ·2001-07-00 ·Pages 307-18

Corcoran RB, Scott MP

Abstract

Medulloblastoma (MB), a tumor of the cerebellum, is the most frequent type of malignant childhood brain tumor. Multiple genes are causally involved in medulloblastoma including PATCHED1 (PTCH). The Patchedl (Ptc1) protein is a receptor for Sonic hedgehog (Shh), a secreted protein ligand. Shh is involved in many signaling processes that control cell fate and growth, among which is its emission from Purkinje cells in the developing cerebellum. Purkinje cell-derived Shh stimulates mitosis of the granule cell precursors that may be the cell type of origin in medulloblastoma. Ptc1 limits the effects of the Shh signal, so mutations in PTCH may lead to persistent granule cell precursors susceptible to further genetic or environmental events that cause medulloblastoma. Mice heterozygous for patched (ptc1) mutations, like heterozygous PTCH humans, have a high rate of medulloblastoma as well as other tumors. We discuss features of the mouse model and how it is contributing to understanding the process of brain tumorigenesis.

MeSH Terms
Animals Basal Cell Nevus Syndrome/genetics Cerebellar Neoplasms/genetics Disease Models, Animal Intracellular Signaling Peptides and Proteins Medulloblastoma/genetics Membrane Proteins/genetics Mice Patched Receptors Patched-1 Receptor Receptors, Cell Surface
Chemicals
Intracellular Signaling Peptides and Proteins Membrane Proteins PTCH1 protein, human Patched Receptors Patched-1 Receptor Ptch1 protein, mouse Receptors, Cell Surface
Authors & Affiliations
2 authors, click to expand affiliations / ORCID
Corcoran R B
Department of Developmental Biology, Howard Hughes Medical Institute, Beckman Center, Stanford University School of Medicine, CA, USA.
Scott M P
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Article Info
Journal
Journal of neuro-oncology
Abbr.
J Neurooncol
ISSN
0167-594X
DOI
10.1023/a:1012260318979
Published
2001-07-00
Pages
307-18
Language
English
Region
United States
NLM ID
8309335
Subset
IM
Grants
Howard Hughes Medical Institute · United States
External Links
PubMed source DOI: 10.1023/a:1012260318979 View journal details
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