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Pedican
Pediatric cancer database
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Basic Information

Gene ID

474

Name

ATOH1

Synonymous

ATH1|HATH1|MATH-1|bHLHa14;atonal homolog 1 (Drosophila);ATOH1;atonal homolog 1 (Drosophila)

Definition

class A basic helix-loop-helix protein 14|helix-loop-helix protein hATH-1|protein atonal homolog 1

Position

4q22

Gene type

protein-coding

Cancer type

Abstract

medulloblastoma;Neurological

Medulloblastoma is the most common malignant pediatric brain tumor. In mice, Ptc1 haploinsufficiency and disruption of DNA repair (DNA ligase IV inactivation) or cell cycle regulation (Kip1, Ink4d, or Ink4c inactivation), in conjunction with p53 dysfunction, predispose to medulloblastoma. To identify genes important for this tumor, we evaluated gene expression profiles in medulloblastomas from thesemice. Unexpectedly, medulloblastoma expression profiles were very similar among tumors and also to those of developing cerebellum. However, 21 genes were specifically up-regulated in medulloblastoma, including sFrp1, Ptc2, and Math1, members of signaling pathways that regulate cerebellar development. Coordinated deregulation of these same genes also occurred in a large subset of human medulloblastomas. These data identify a group of genes that is central to medulloblastoma tumorigenesis.

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