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Gene Review

Atoh1  -  atonal homolog 1 (Drosophila)

Mus musculus

Synonyms: Ath1, Hath1, Helix-loop-helix protein mATH-1, MATH-1, Math1, ...
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Disease relevance of Atoh1


High impact information on Atoh1


Biological context of Atoh1


Anatomical context of Atoh1

  • The transcription factor Math1 (encoded by the gene Atoh1, also called Math1) is required for the formation of mechanosensory hair cells in the inner ear; however, its specific molecular role is unknown [11].
  • It is thought that small intestinal epithelial stem cell progeny, via Notch signaling, yield a Hes1-expressing columnar lineage progenitor and an Atoh1 (also known as Math1)-expressing common progenitor for all granulocytic lineages including enteroendocrine cells, one of the body's largest populations of endocrine cells [12].
  • E18.5 Atoh1 null mice have many afferent fibers to the apex of the cochlea, the anterior and the posterior crista, all areas with numerous Bdnf-beta-galactosidase-positive cells [13].
  • Absence of Bdnf-beta-galactosidase expression in the utricle and saccule of Atoh1 null mice is first noted at E12.5, a time when Atoh1-beta-galactosidase expression is also first detected in these epithelia [13].
  • Inhibitors of differentiation and DNA binding (Ids) regulate Math1 and hair cell formation during the development of the organ of Corti [14].

Associations of Atoh1 with chemical compounds


Physical interactions of Atoh1


Regulatory relationships of Atoh1

  • Therefore, Id proteins are good candidates for regulating Math1 in the cochlea [14].
  • The Barhl1 expression has been demonstrated to be induced by the transcription factor Math1 involved in BMP responses [17].
  • These results are consistent with the hypothesis that activation of Notch via Jagged2 acts to inhibit expression of Math1 in cochlear progenitor cells, possibly through the activity of HES5 [18].
  • Generation of commissural neurons by Math1 was inhibited by a dominant negative form of Mbh1 [16].

Other interactions of Atoh1

  • The bHLH-related inhibitors of differentiation and DNA binding (Id) proteins are known to negatively regulate many bHLH transcription factors, including Math1, in a number of different systems [14].
  • These results indicate a key role for Ids in the regulation of expression of Math1 and hair cell differentiation in the developing cochlea [14].
  • The developmental expression pattern of Fgf14 in the cerebellum is complementary to that of Math1, a marker for proliferating granule cells in the external germinal layer [19].
  • Neurotrophins facilitate neuronal differentiation of cultured neural stem cells via induction of mRNA expression of basic helix-loop-helix transcription factors Mash1 and Math1 [20].
  • Some of the dorsal Olig3 lineage cells co-expressed Islet1/2, Math1, or Brn3a, markers for dorsal interneuron [21].

Analytical, diagnostic and therapeutic context of Atoh1

  • After purification by FACS and coculture with postnatal cerebellar neurons, ES cell-derived Math1+ cells exhibit typical features of neurons of the external granule cell layer, including extensive motility and a T-shaped morphology [22].
  • 5. PCR analysis shows expression of Atoh 1 in the otocyst as early as E10.5, prior to any histology-based detection techniques [23].
  • A similar expression of Atoh 1-LacZ exists in postnatal and adult vestibular and cochlear sensory neurons, and Atoh 1 expression in vestibular sensory neurons is confirmed with RT-PCR [23].
  • Dissection of the cellular and molecular events that position cerebellar Purkinje cells: a study of the math1 null-mutant mouse [24].
  • Taken together, we propose that when putative direct targets of Math1 are being selected for detailed studies on DNA microarray hybridization, the enrichment and clustering of binding E-boxes in multiple species may be helpful criteria [15].


  1. A molecular fingerprint for medulloblastoma. Lee, Y., Miller, H.L., Jensen, P., Hernan, R., Connelly, M., Wetmore, C., Zindy, F., Roussel, M.F., Curran, T., Gilbertson, R.J., McKinnon, P.J. Cancer Res. (2003) [Pubmed]
  2. A mammalian helix-loop-helix factor structurally related to the product of Drosophila proneural gene atonal is a positive transcriptional regulator expressed in the developing nervous system. Akazawa, C., Ishibashi, M., Shimizu, C., Nakanishi, S., Kageyama, R. J. Biol. Chem. (1995) [Pubmed]
  3. A Role for Hath1, a bHLH Transcription Factor, in Colon Adenocarcinoma. Leow, C.C., Polakis, P., Gao, W.Q. Ann. N. Y. Acad. Sci. (2005) [Pubmed]
  4. Requirement of Math1 for secretory cell lineage commitment in the mouse intestine. Yang, Q., Bermingham, N.A., Finegold, M.J., Zoghbi, H.Y. Science (2001) [Pubmed]
  5. Math1: an essential gene for the generation of inner ear hair cells. Bermingham, N.A., Hassan, B.A., Price, S.D., Vollrath, M.A., Ben-Arie, N., Eatock, R.A., Bellen, H.J., Lysakowski, A., Zoghbi, H.Y. Science (1999) [Pubmed]
  6. Gfi1 functions downstream of Math1 to control intestinal secretory cell subtype allocation and differentiation. Shroyer, N.F., Wallis, D., Venken, K.J., Bellen, H.J., Zoghbi, H.Y. Genes Dev. (2005) [Pubmed]
  7. Math1 controls cerebellar granule cell differentiation by regulating multiple components of the Notch signaling pathway. Gazit, R., Krizhanovsky, V., Ben-Arie, N. Development (2004) [Pubmed]
  8. Inhibition of Notch/RBP-J signaling induces hair cell formation in neonate mouse cochleas. Yamamoto, N., Tanigaki, K., Tsuji, M., Yabe, D., Ito, J., Honjo, T. J. Mol. Med. (2006) [Pubmed]
  9. Hath1 up-regulates gastric mucin gene expression in gastric cells. Sekine, A., Akiyama, Y., Yanagihara, K., Yuasa, Y. Biochem. Biophys. Res. Commun. (2006) [Pubmed]
  10. Math1 is expressed in temporally discrete pools of cerebellar rhombic-lip neural progenitors. Machold, R., Fishell, G. Neuron (2005) [Pubmed]
  11. Math1 regulates development of the sensory epithelium in the mammalian cochlea. Woods, C., Montcouquiol, M., Kelley, M.W. Nat. Neurosci. (2004) [Pubmed]
  12. Neurogenin 3 and the enteroendocrine cell lineage in the adult mouse small intestinal epithelium. Bjerknes, M., Cheng, H. Dev. Biol. (2006) [Pubmed]
  13. Atoh1 null mice show directed afferent fiber growth to undifferentiated ear sensory epithelia followed by incomplete fiber retention. Fritzsch, B., Matei, V.A., Nichols, D.H., Bermingham, N., Jones, K., Beisel, K.W., Wang, V.Y. Dev. Dyn. (2005) [Pubmed]
  14. Inhibitors of differentiation and DNA binding (Ids) regulate Math1 and hair cell formation during the development of the organ of Corti. Jones, J.M., Montcouquiol, M., Dabdoub, A., Woods, C., Kelley, M.W. J. Neurosci. (2006) [Pubmed]
  15. Math1 Target Genes Are Enriched With Evolutionarily Conserved Clustered E-box Binding Sites. Krizhanovsky, V., Soreq, L., Kliminski, V., Ben-Arie, N. J. Mol. Neurosci. (2006) [Pubmed]
  16. Commissural neuron identity is specified by a homeodomain protein, Mbh1, that is directly downstream of Math1. Saba, R., Johnson, J.E., Saito, T. Development (2005) [Pubmed]
  17. Differential transcription of Barhl1 homeobox gene in restricted functional domains of the central nervous system suggests a role in brain patterning. Rachidi, M., Lopes, C. Int. J. Dev. Neurosci. (2006) [Pubmed]
  18. Expression of Math1 and HES5 in the cochleae of wildtype and Jag2 mutant mice. Lanford, P.J., Shailam, R., Norton, C.R., Gridley, T., Kelley, M.W. J. Assoc. Res. Otolaryngol. (2000) [Pubmed]
  19. Subcellular and developmental expression of alternatively spliced forms of fibroblast growth factor 14. Wang, Q., McEwen, D.G., Ornitz, D.M. Mech. Dev. (2000) [Pubmed]
  20. Neurotrophins facilitate neuronal differentiation of cultured neural stem cells via induction of mRNA expression of basic helix-loop-helix transcription factors Mash1 and Math1. Ito, H., Nakajima, A., Nomoto, H., Furukawa, S. J. Neurosci. Res. (2003) [Pubmed]
  21. Short-term lineage analysis of dorsally derived Olig3 cells in the developing spinal cord. Ding, L., Takebayashi, H., Watanabe, K., Ohtsuki, T., Tanaka, K.F., Nabeshima, Y., Chisaka, O., Ikenaka, K., Ono, K. Dev. Dyn. (2005) [Pubmed]
  22. Generation of cerebellar neuron precursors from embryonic stem cells. Su, H.L., Muguruma, K., Matsuo-Takasaki, M., Kengaku, M., Watanabe, K., Sasai, Y. Dev. Biol. (2006) [Pubmed]
  23. Smaller inner ear sensory epithelia in Neurog 1 null mice are related to earlier hair cell cycle exit. Matei, V., Pauley, S., Kaing, S., Rowitch, D., Beisel, K.W., Morris, K., Feng, F., Jones, K., Lee, J., Fritzsch, B. Dev. Dyn. (2005) [Pubmed]
  24. Dissection of the cellular and molecular events that position cerebellar Purkinje cells: a study of the math1 null-mutant mouse. Jensen, P., Zoghbi, H.Y., Goldowitz, D. J. Neurosci. (2002) [Pubmed]
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