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Atonal homolog 1 (Drosophila)
Symbols ATOH1; ATH1; HATH1; MATH-1
External IDs OMIM601461 MGI104654 HomoloGene31297 GeneCards: ATOH1 Gene
RNA expression pattern
PBB GE ATOH1 221336 at tn.png
More reference expression data
Species Human Mouse
Entrez 474 11921
Ensembl ENSG00000172238 ENSMUSG00000073043
UniProt Q92858 P48985
RefSeq (mRNA) NM_005172 NM_007500
RefSeq (protein) NP_005163 NP_031526
Location (UCSC) Chr 4:
94.97 - 94.97 Mb
Chr 6:
64.66 - 64.66 Mb
PubMed search [1] [2]

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 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. PMID 8872459.  
  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. PMID 9449674.  
  • 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. doi:10.1074/jbc.M108417200. PMID 11748221.  
  • 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. doi:10.1007/s00335-001-2101-3. PMID 11889557.  
  • 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. doi:10.1002/ijc.10554. PMID 12209986.  
  • 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. doi:10.1073/pnas.242603899. PMID 12477932.  
  • 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. doi:10.1242/dev.00982. PMID 14757642.  
  • 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. doi:10.1101/gr.2596504. PMID 15489334.  
  • 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. doi:10.1196/annals.1339.048. PMID 16382053.  
  • 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. doi:10.1016/j.bbrc.2006.03.238. PMID 16647036.  
  • 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. doi:10.1111/j.1432-0436.2006.00074.x. PMID 16831200.  
  • 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. doi:10.1158/1078-0432.CCR-06-0573. PMID 17000673.  
  • 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. doi:10.1053/j.gastro.2006.10.031. PMID 17241872.  
  • 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. doi:10.1016/j.ydbio.2007.02.028. PMID 17397818.  
  • 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. PMID 17549667.  
  • 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. doi:10.1016/j.febslet.2007.08.059. PMID 17826772.  
  • 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. doi:10.1053/j.gastro.2007.03.047. PMID 17570220.  

External links

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



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