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Title: Atoh1  
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Subject: HES1, Transcription factors, NeuroD, EMX homeogene, Engrailed (gene)
Collection: Transcription Factors
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Atonal homolog 1 (Drosophila)
Symbols  ; ATH1; HATH1; MATH-1; bHLHa14
External IDs GeneCards:
RNA expression pattern
Species Human Mouse
RefSeq (mRNA)
RefSeq (protein)
Location (UCSC)
PubMed search

Protein atonal homolog 1 is a protein that in humans is encoded by the ATOH1 gene.[1][2] This protein belongs to the basic helix-loop-helix (BHLH) family of transcription factors. It activates E-box dependent transcription along with E47.[2] ATOH1 is required for the formation of both neural and non-neural cell types. Using genetic deletion in mice, Atoh1 has been shown to be essential for formation of cerebellar granule neurons, inner ear hair cells, spinal cord interneurons, Merkel cells of the skin, and intestinal secretory cells (goblet, enteroendocrine, and Paneth cells). ATOH1 is a mammalian homolog of the Drosophila melanogaster gene atonal. ATOH1 is considered part of the Notch signaling pathway.

In 2009, ATOH1 was identified as a "cancer switch-off" gene. [3]


  1. ^ Ben-Arie N, McCall AE, Berkman S, Eichele G, Bellen HJ, Zoghbi HY (Jan 1997). "Evolutionary conservation of sequence and expression of the bHLH protein Atonal suggests a conserved role in neurogenesis". Hum Mol Genet 5 (9): 1207–16.  
  2. ^ a b "Entrez Gene: ATOH1 atonal homolog 1 (Drosophila)". 

Further reading

  • Helms AW, Johnson JE (1998). "Progenitors of dorsal commissural interneurons are defined by MATH1 expression". Development 125 (5): 919–28.  
  • Dintilhac A, Bernués J (2002). "HMGB1 interacts with many apparently unrelated proteins by recognizing short amino acid sequences". J. Biol. Chem. 277 (9): 7021–8.  
  • Brown NL, Dagenais SL, Chen CM, Glaser T (2002). "Molecular characterization and mapping of ATOH7, a human atonal homolog with a predicted role in retinal ganglion cell development". Mamm. Genome 13 (2): 95–101.  
  • Leonard JH, Cook AL, Van Gele M, et al. (2002). "Proneural and proneuroendocrine transcription factor expression in cutaneous mechanoreceptor (Merkel) cells and Merkel cell carcinoma". Int. J. Cancer 101 (2): 103–10.  
  • Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903.  
  • Gazit R, Krizhanovsky V, Ben-Arie N (2004). "Math1 controls cerebellar granule cell differentiation by regulating multiple components of the Notch signaling pathway". Development 131 (4): 903–13.  
  • Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The Status, Quality, and Expansion of the NIH Full-Length cDNA Project: The Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–7.  
  • Leow CC, Polakis P, Gao WQ (2006). "A role for Hath1, a bHLH transcription factor, in colon adenocarcinoma". Ann. N. Y. Acad. Sci. 1059: 174–83.  
  • Sekine A, Akiyama Y, Yanagihara K, Yuasa Y (2006). "Hath1 up-regulates gastric mucin gene expression in gastric cells". Biochem. Biophys. Res. Commun. 344 (4): 1166–71.  
  • Mutoh H, Sakamoto H, Hayakawa H, et al. (2006). "The intestine-specific homeobox gene Cdx2 induces expression of the basic helix-loop-helix transcription factor Math1". Differentiation 74 (6): 313–21.  
  • Park ET, Oh HK, Gum JR, et al. (2006). "HATH1 expression in mucinous cancers of the colorectum and related lesions". Clin. Cancer Res. 12 (18): 5403–10.  
  • Tsuchiya K, Nakamura T, Okamoto R, et al. (2007). "Reciprocal targeting of Hath1 and beta-catenin by Wnt glycogen synthase kinase 3beta in human colon cancer". Gastroenterology 132 (1): 208–20.  
  • Du X, Jensen P, Goldowitz D, Hamre KM (2007). "Wild-type cells rescue genotypically Math1-null hair cells in the inner ears of chimeric mice". Dev. Biol. 305 (2): 430–8.  
  • Westerman BA, Breuer RH, Poutsma A, et al. (2007). "Basic helix-loop-helix transcription factor profiling of lung tumors shows aberrant expression of the proneural gene atonal homolog 1 (ATOH1, HATH1, MATH1) in neuroendocrine tumors". Int. J. Biol. Markers 22 (2): 114–23.  
  • Scheffer D, Sage C, Corey DP, Pingault V (2007). "Gene expression profiling identifies Hes6 as a transcriptional target of ATOH1 in cochlear hair cells". FEBS Lett. 581 (24): 4651–6.  
  • Shroyer NF, Helmrath MA, Wang VY, Antalffy B, Henning SJ, Zoghbi HY (2007). "Intestine-specific ablation of mouse atonal homolog 1 (Math1) reveals a role in cellular homeostasis". Gastroenterology 132 (7): 2478–88.  

External links

  • ATOH1 protein, human at the US National Library of Medicine Medical Subject Headings (MeSH)
  • ATOH1 and cancer suppression

This article incorporates text from the United States National Library of Medicine, which is in the public domain.

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