Gene Symbol: Atoh1
Description: atonal bHLH transcription factor 1
Alias: Hath1, MATH-1, Math1, bHLHa14, protein atonal homolog 1, atonal homolog 1, helix-loop-helix protein mATH-1
Species: mouse
Products:     Atoh1

Top Publications

  1. VanDussen K, Samuelson L. Mouse atonal homolog 1 directs intestinal progenitors to secretory cell rather than absorptive cell fate. Dev Biol. 2010;346:215-23 pubmed publisher
    The Notch-regulated transcription factor mouse atonal homolog 1 (Math1) is required for the development of intestinal secretory cells, as demonstrated by the loss of goblet, endocrine and Paneth cell types in null mice...
  2. Shroyer N, Helmrath M, Wang V, Antalffy B, Henning S, Zoghbi H. Intestine-specific ablation of mouse atonal homolog 1 (Math1) reveals a role in cellular homeostasis. Gastroenterology. 2007;132:2478-88 pubmed
    Math1 (Atoh1) is a basic helix-loop-helix transcription factor important for intestinal secretory cell differentiation...
  3. Kiesslich R, Goetz M, Angus E, Hu Q, Guan Y, Potten C, et al. Identification of epithelial gaps in human small and large intestine by confocal endomicroscopy. Gastroenterology. 2007;133:1769-78 pubmed
    ..The goals of this study were to determine if confocal endomicroscopy could resolve the presence of human epithelial gaps and whether a proinflammatory cytokine could increase cell shedding...
  4. Doetzlhofer A, Basch M, Ohyama T, Gessler M, Groves A, Segil N. Hey2 regulation by FGF provides a Notch-independent mechanism for maintaining pillar cell fate in the organ of Corti. Dev Cell. 2009;16:58-69 pubmed publisher
  5. Bossuyt W, Kazanjian A, De Geest N, Van Kelst S, De Hertogh G, Geboes K, et al. Atonal homolog 1 is a tumor suppressor gene. PLoS Biol. 2009;7:e39 pubmed publisher
    ..Our genetic evidence from Drosophila and previous in vitro studies of mammalian Atonal homolog 1 (Atoh1, also called Math1 or Hath1) suggest an anti-oncogenic function for the Atonal group of proneural ..
  6. Helms A, Johnson J. Progenitors of dorsal commissural interneurons are defined by MATH1 expression. Development. 1998;125:919-28 pubmed
    b>MATH1 is a neural-specific basic helix-loop-helix transcription factor. Members of this family of transcription factors are involved in the development of specific subsets of neurons in the developing vertebrate nervous system...
  7. Munnamalai V, Hayashi T, Bermingham McDonogh O. Notch prosensory effects in the Mammalian cochlea are partially mediated by Fgf20. J Neurosci. 2012;32:12876-84 pubmed publisher
    ..We hypothesized that Fgf20 plays a role in specification, amplification, or maintenance of Sox2 expression in prosensory progenitors of the developing mammalian cochlea...
  8. Hayashi T, Ray C, Bermingham McDonogh O. Fgf20 is required for sensory epithelial specification in the developing cochlea. J Neurosci. 2008;28:5991-9 pubmed publisher increases in cell death or changes in proliferation but lead to a rapid reduction in Pea3 and Erm and a loss of Math1 expression...
  9. Wallis D, Hamblen M, Zhou Y, Venken K, Schumacher A, Grimes H, et al. The zinc finger transcription factor Gfi1, implicated in lymphomagenesis, is required for inner ear hair cell differentiation and survival. Development. 2003;130:221-32 pubmed
    ..Hence, Gfi1 is expressed in the developing nervous system, is required for inner ear hair cell differentiation, and its loss causes programmed cell death. ..

More Information


  1. Hagan N, Zervas M. Wnt1 expression temporally allocates upper rhombic lip progenitors and defines their terminal cell fate in the cerebellum. Mol Cell Neurosci. 2012;49:217-29 pubmed publisher
    ..molecular map of Wnt1 expressing progenitors in relation to other well characterized Cb biomarkers such as MATH1 (ATOH1), LMX1a and OTX2...
  2. Yu T, Yaguchi Y, Echevarria D, Martinez S, Basson M. Sprouty genes prevent excessive FGF signalling in multiple cell types throughout development of the cerebellum. Development. 2011;138:2957-68 pubmed publisher
    ..Taken together, our data demonstrate that FGF signalling levels have to be tightly controlled throughout cerebellar development in order to maintain the normal development of multiple cell types. ..
  3. Kazanjian A, Noah T, Brown D, Burkart J, Shroyer N. Atonal homolog 1 is required for growth and differentiation effects of notch/gamma-secretase inhibitors on normal and cancerous intestinal epithelial cells. Gastroenterology. 2010;139:918-28, 928.e1-6 pubmed publisher
    The atonal homolog 1 (Atoh1) transcription factor is required for intestinal secretory (goblet, Paneth, enteroendocrine) cell differentiation...
  4. Weston M, Pierce M, Jensen Smith H, Fritzsch B, Rocha Sanchez S, Beisel K, et al. MicroRNA-183 family expression in hair cell development and requirement of microRNAs for hair cell maintenance and survival. Dev Dyn. 2011;240:808-19 pubmed publisher
    ..Depletion of hair cell miRNAs resulting from Dicer1 conditional knockout (CKO) in Atoh1-Cre transgenic mice leads to more disparate basal-apical gene expression profiles and eventual hair cell loss...
  5. Jensen P, Smeyne R, Goldowitz D. Analysis of cerebellar development in math1 null embryos and chimeras. J Neurosci. 2004;24:2202-11 pubmed
    ..The earliest known gene to be expressed in the development of these cells is math1. In the math1 null mouse, neuroblasts never populate the external germinal layer (EGL) that gives rise to granule ..
  6. Driver E, Sillers L, Coate T, Rose M, Kelley M. The Atoh1-lineage gives rise to hair cells and supporting cells within the mammalian cochlea. Dev Biol. 2013;376:86-98 pubmed publisher
    ..early stages of cochlear development, and both HCs and SCs are absent in mice lacking the transcription factor Atoh1. Based on existing data, Atoh1 is thought to be the earliest determinant of HC fate, and to have a cell-autonomous ..
  7. Cox B, Liu Z, Lagarde M, Zuo J. Conditional gene expression in the mouse inner ear using Cre-loxP. J Assoc Res Otolaryngol. 2012;13:295-322 pubmed publisher
    ..We compare the Cre expression patterns between Atoh1-CreER(TM) and Atoh1-CreER(T2) and report a new line, Fgfr3-iCreER(T2), which displays inducible Cre activity in ..
  8. Flora A, Garcia J, Thaller C, Zoghbi H. The E-protein Tcf4 interacts with Math1 to regulate differentiation of a specific subset of neuronal progenitors. Proc Natl Acad Sci U S A. 2007;104:15382-7 pubmed
    ..To test this hypothesis we isolated proteins that interact with Math1, a proneural transcription factor essential for the establishment of a neural progenitor population (rhombic lip) ..
  9. Millonig J, Millen K, Hatten M. The mouse Dreher gene Lmx1a controls formation of the roof plate in the vertebrate CNS. Nature. 2000;403:764-9 pubmed
    ..Lmx1a is expressed in the roof plate along the neuraxis during development of the CNS. Thus, Lmx1a is required for development of the roof plate and, in turn, for specification of dorsal cell fates in the CNS and developing vertebrae. ..
  10. Akazawa C, Ishibashi M, Shimizu C, Nakanishi S, Kageyama R. 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. J Biol Chem. 1995;270:8730-8 pubmed
    ..These results suggest that MATH-1 may be a target of HES-1 and play a role in the differentiation of subsets of neural cells by activating E box-dependent transcription. ..
  11. Cai T, Seymour M, Zhang H, Pereira F, Groves A. Conditional deletion of Atoh1 reveals distinct critical periods for survival and function of hair cells in the organ of Corti. J Neurosci. 2013;33:10110-22 pubmed publisher
    Atonal homolog1 (Atoh1) encodes a basic helix-loop-helix protein that is the first transcription factor to be expressed in differentiating hair cells...
  12. Maricich S, Morrison K, Mathes E, Brewer B. Rodents rely on Merkel cells for texture discrimination tasks. J Neurosci. 2012;32:3296-300 pubmed publisher
    ..These findings suggest that Merkel cell/neurite complexes are essential for texture discrimination tasks involving glabrous skin but not whiskers. ..
  13. Ramanantsoa N, Hirsch M, Thoby Brisson M, Dubreuil V, Bouvier J, Ruffault P, et al. Breathing without CO(2) chemosensitivity in conditional Phox2b mutants. J Neurosci. 2011;31:12880-8 pubmed publisher
    ..Hence, during the early life of rodents, the excitatory input normally afforded by elevated CO(2) is dispensable for life-sustaining breathing and maintaining CO(2) homeostasis in the blood. ..
  14. Gregorieff A, Stange D, Kujala P, Begthel H, van den Born M, Korving J, et al. The ets-domain transcription factor Spdef promotes maturation of goblet and paneth cells in the intestinal epithelium. Gastroenterology. 2009;137:1333-45.e1-3 pubmed publisher
    ..In this study, we investigated the role of the Wnt-responsive, Ets-domain transcription factor Spdef in the differentiation of goblet and Paneth cells...
  15. Srivastava R, Kumar M, Peineau S, Csaba Z, Mani S, Gressens P, et al. Conditional induction of Math1 specifies embryonic stem cells to cerebellar granule neuron lineage and promotes differentiation into mature granule neurons. Stem Cells. 2013;31:652-65 pubmed publisher
    ..we have generated mature cerebellar granule neurons by combining a temporally controlled transient expression of Math1, a master gene in granule neuron differentiation, with inductive extrinsic factors involved in cerebellar ..
  16. Liu Z, Owen T, Fang J, Srinivasan R, Zuo J. In vivo Notch reactivation in differentiating cochlear hair cells induces Sox2 and Prox1 expression but does not disrupt hair cell maturation. Dev Dyn. 2012;241:684-96 pubmed publisher
    ..By analyzing Atoh1(CreER+) ; Rosa26-NICD(loxp/+) or Atoh1(CreER+) ; Rosa26-NICD(loxp/+) ; RBP-J(loxp/loxp) mice, we demonstrated that ..
  17. Li S, Qiu F, Xu A, Price S, Xiang M. Barhl1 regulates migration and survival of cerebellar granule cells by controlling expression of the neurotrophin-3 gene. J Neurosci. 2004;24:3104-14 pubmed
    ..These results suggest that Barhl1 plays an essential role in the migration and survival of cerebellar granule cells and precerebellar neurons and functionally link Barhl1 to the NT-3 signaling pathway during cerebellar development. ..
  18. Raft S, Koundakjian E, Quinones H, Jayasena C, Goodrich L, Johnson J, et al. Cross-regulation of Ngn1 and Math1 coordinates the production of neurons and sensory hair cells during inner ear development. Development. 2007;134:4405-15 pubmed
    ..The related bHLH genes Ngn1 (Neurog1) and Math1 (Atoh1) are required, respectively, for neural and sensory epithelial development in this system...
  19. Izumikawa M, Batts S, Miyazawa T, Swiderski D, Raphael Y. Response of the flat cochlear epithelium to forced expression of Atoh1. Hear Res. 2008;240:52-6 pubmed publisher
    ..Atoh1 (previously Math1) is a basic helix-loop-helix transcription factor critical to hair cell differentiation during mammalian ..
  20. Kelly M, Chang Q, Pan A, Lin X, Chen P. Atoh1 directs the formation of sensory mosaics and induces cell proliferation in the postnatal mammalian cochlea in vivo. J Neurosci. 2012;32:6699-710 pubmed publisher
    ..Considerable interest targets the hair cell differentiation factor Atoh1 as a potential tool with which to promote hair cell regeneration...
  21. Fujiyama T, Yamada M, Terao M, Terashima T, Hioki H, Inoue Y, et al. Inhibitory and excitatory subtypes of cochlear nucleus neurons are defined by distinct bHLH transcription factors, Ptf1a and Atoh1. Development. 2009;136:2049-58 pubmed publisher
    ..Pancreatic transcription factor 1a (Ptf1a) and atonal homolog 1 (Atoh1) were found to be expressed in discrete dorsolateral regions of the embryonic neuroepithelia of the ..
  22. Wang V, Hassan B, Bellen H, Zoghbi H. Drosophila atonal fully rescues the phenotype of Math1 null mice: new functions evolve in new cellular contexts. Curr Biol. 2002;12:1611-6 pubmed
    ..We have been analyzing the relationship between atonal (ato) in the fruit fly and its mouse homolog, Math1. In flies, ato acts as a proneural gene that governs the development of chordotonal organs (CHOs), which serve as ..
  23. Machold R, Fishell G. Math1 is expressed in temporally discrete pools of cerebellar rhombic-lip neural progenitors. Neuron. 2005;48:17-24 pubmed
    We have utilized an in vivo-inducible genetic-fate-mapping strategy to permanently label cohorts of Math1-positive cells and their progeny that arise in the rhombic lip of the cerebellar primordium during embryogenesis...
  24. Ben Arie N, Bellen H, Armstrong D, McCall A, Gordadze P, Guo Q, et al. Math1 is essential for genesis of cerebellar granule neurons. Nature. 1997;390:169-72 pubmed
    ..b>Math1, the mouse homologue of the Drosophila gene atonal, encodes a basic helix-loop-helix transcription factor that is ..
  25. Chizhikov V, Lindgren A, Currle D, Rose M, Monuki E, Millen K. The roof plate regulates cerebellar cell-type specification and proliferation. Development. 2006;133:2793-804 pubmed
    ..Thus, in addition to the isthmus, the roof plate represents an important signaling center controlling multiple aspects of cerebellar patterning. ..
  26. Gowan K, Helms A, Hunsaker T, Collisson T, Ebert P, Odom R, et al. Crossinhibitory activities of Ngn1 and Math1 allow specification of distinct dorsal interneurons. Neuron. 2001;31:219-32 pubmed
    ..We define restricted neural progenitor populations by the discrete, nonoverlapping expression of Ngn1, Math1, and Mash1...
  27. Pan N, Jahan I, Kersigo J, Duncan J, Kopecky B, Fritzsch B. A novel Atoh1 "self-terminating" mouse model reveals the necessity of proper Atoh1 level and duration for hair cell differentiation and viability. PLoS ONE. 2012;7:e30358 pubmed publisher
    Atonal homolog1 (Atoh1) is a bHLH transcription factor essential for inner ear hair cell differentiation. Targeted expression of Atoh1 at various stages in development can result in hair cell differentiation in the ear...
  28. Chen P, Zindy F, Abdala C, Liu F, Li X, Roussel M, et al. Progressive hearing loss in mice lacking the cyclin-dependent kinase inhibitor Ink4d. Nat Cell Biol. 2003;5:422-6 pubmed
    ..Our results identify a novel mechanism underlying a non-syndromic form of progressive hearing loss in mice. ..
  29. Lütolf S, Radtke F, Aguet M, Suter U, Taylor V. Notch1 is required for neuronal and glial differentiation in the cerebellum. Development. 2002;129:373-85 pubmed
    ..We have also analyzed the effects of Notch1 ablation on gliogenesis in vivo. Our results show that Notch1 is required for both neuron and glia formation and modulates the onset of neurogenesis within the cerebellar neuroepithelium. ..
  30. Hu X, Huang J, Feng L, Fukudome S, Hamajima Y, Lin J. Sonic hedgehog (SHH) promotes the differentiation of mouse cochlear neural progenitors via the Math1-Brn3.1 signaling pathway in vitro. J Neurosci Res. 2010;88:927-35 pubmed publisher
    ..of SHH to CNPs increased the formation of epithelial cell islands, simultaneously activated the expression of Math1 that is a transcription factor for the initial differentiation of auditory hair cells...
  31. Ahmed M, Wong E, Sun J, Xu J, Wang F, Xu P. Eya1-Six1 interaction is sufficient to induce hair cell fate in the cochlea by activating Atoh1 expression in cooperation with Sox2. Dev Cell. 2012;22:377-90 pubmed publisher
    Inner-ear hair cell differentiation requires Atoh1 function, while Eya1, Six1, and Sox2 are coexpressed in sensory progenitors and mutations in these genes cause sensorineural hearing loss...
  32. Bermingham N, Hassan B, Price S, Vollrath M, Ben Arie N, Eatock R, et al. Math1: an essential gene for the generation of inner ear hair cells. Science. 1999;284:1837-41 pubmed
    ..The molecular mechanisms underlying hair cell development and differentiation are poorly understood. Math1, a mouse homolog of the Drosophila proneural gene atonal, is expressed in inner ear sensory epithelia...
  33. Pan N, Jahan I, Lee J, Fritzsch B. Defects in the cerebella of conditional Neurod1 null mice correlate with effective Tg(Atoh1-cre) recombination and granule cell requirements for Neurod1 for differentiation. Cell Tissue Res. 2009;337:407-28 pubmed publisher
    ..gene for most cerebellar granule cells and mediates the differentiation of these cells downstream of Atoh1-mediated proliferation of the precursors...
  34. Chen P, Johnson J, Zoghbi H, Segil N. The role of Math1 in inner ear development: Uncoupling the establishment of the sensory primordium from hair cell fate determination. Development. 2002;129:2495-505 pubmed
    ..b>Math1, a close homolog of the Drosophila proneural gene atonal, has been found to be both necessary and sufficient for ..
  35. Yang Q, Bermingham N, Finegold M, Zoghbi H. Requirement of Math1 for secretory cell lineage commitment in the mouse intestine. Science. 2001;294:2155-8 pubmed
    ..Previous studies showed that Math1, a basic helix-loop-helix (bHLH) transcription factor, is expressed in the gut...
  36. Kiernan A, Pelling A, Leung K, Tang A, Bell D, Tease C, et al. Sox2 is required for sensory organ development in the mammalian inner ear. Nature. 2005;434:1031-5 pubmed
    ..SOX2 continues to be expressed in the inner ears of mice lacking Math1 (also known as Atoh1 and HATH1), a gene essential for hair cell differentiation, whereas Math1 expression is absent in Lcc mutants, suggesting ..
  37. Kawauchi D, Saito T. Transcriptional cascade from Math1 to Mbh1 and Mbh2 is required for cerebellar granule cell differentiation. Dev Biol. 2008;322:345-54 pubmed publisher
    ..neuronal type in the mammalian brain, and their differentiation is regulated by the basic helix-loop-helix gene, Math1. However, little is known about downstream genes of Math1 and their functions in the cerebellum...
  38. Miesegaes G, Klisch T, Thaller C, Ahmad K, Atkinson R, Zoghbi H. Identification and subclassification of new Atoh1 derived cell populations during mouse spinal cord development. Dev Biol. 2009;327:339-51 pubmed publisher
    ..In the past we revealed the basic helix-loop-helix transcription factor Atoh1 (Math1) to be important for establishing Dorsal Progenitor 1 (DP1) commissural interneurons, which comprise a subset of ..
  39. Jahan I, Pan N, Kersigo J, Fritzsch B. Neurod1 suppresses hair cell differentiation in ear ganglia and regulates hair cell subtype development in the cochlea. PLoS ONE. 2010;5:e11661 pubmed publisher
    ..Cross-regulation of Atoh1 and Neurog1 results in hair cell changes in Neurog1 null mice although the nature and mechanism of the cross-..
  40. Lewis P, Gritli Linde A, Smeyne R, Kottmann A, McMahon A. Sonic hedgehog signaling is required for expansion of granule neuron precursors and patterning of the mouse cerebellum. Dev Biol. 2004;270:393-410 pubmed
    ..Together, these findings provide additional evidence for Shh's key regulatory role in controlling growth of the cerebellar primordium. ..
  41. Hertzano R, Montcouquiol M, Rashi Elkeles S, Elkon R, Yücel R, Frankel W, et al. Transcription profiling of inner ears from Pou4f3(ddl/ddl) identifies Gfi1 as a target of the Pou4f3 deafness gene. Hum Mol Genet. 2004;13:2143-53 pubmed
    ..These results identify Gfi1 as the first downstream target of a hair cell specific transcription factor and suggest that outer hair cell degeneration in Pou4f3 mutants is largely or entirely a result of the loss of expression of Gfi1. ..
  42. Chizhikov V, Lindgren A, Mishima Y, Roberts R, Aldinger K, Miesegaes G, et al. Lmx1a regulates fates and location of cells originating from the cerebellar rhombic lip and telencephalic cortical hem. Proc Natl Acad Sci U S A. 2010;107:10725-30 pubmed publisher
    ..Thus, Lmx1a activity is critical for proper production of cells originating from both the cerebellar rhombic lip and the telencephalic cortical hem. ..
  43. Yamamoto N, Chang W, Kelley M. Rbpj regulates development of prosensory cells in the mammalian inner ear. Dev Biol. 2011;353:367-79 pubmed publisher
    ..developing cochleae in Rbpj-mutants indicates changes in the spatiotemporal patterns of expression for p27(kip1), Atoh1 and hair cell differentiation markers implicating notch signaling in the regulation of the timing of cellular ..
  44. Pirvola U, Ylikoski J, Trokovic R, Hebert J, McConnell S, Partanen J. FGFR1 is required for the development of the auditory sensory epithelium. Neuron. 2002;35:671-80 pubmed
    ..Our data also suggest that FGFR1 might have a distinct later role in intercellular signaling within the differentiating auditory sensory epithelium. ..
  45. Kim T, Shivdasani R. Genetic evidence that intestinal Notch functions vary regionally and operate through a common mechanism of Math1 repression. J Biol Chem. 2011;286:11427-33 pubmed publisher
    ..Pharmacologic studies suggest that the latter effect occurs through the transcription factor Math1/Atoh1, which specifies all intestinal secretory cells. We tested this hypothesis using mouse mutants...
  46. Tateya T, Imayoshi I, Tateya I, Ito J, Kageyama R. Cooperative functions of Hes/Hey genes in auditory hair cell and supporting cell development. Dev Biol. 2011;352:329-40 pubmed publisher
  47. Woods C, Montcouquiol M, Kelley M. Math1 regulates development of the sensory epithelium in the mammalian cochlea. Nat Neurosci. 2004;7:1310-8 pubmed
    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...
  48. Liu Z, Dearman J, Cox B, Walters B, Zhang L, Ayrault O, et al. Age-dependent in vivo conversion of mouse cochlear pillar and Deiters' cells to immature hair cells by Atoh1 ectopic expression. J Neurosci. 2012;32:6600-10 pubmed publisher
    ..of two SC subtypes, pillar cells (PCs) and Deiters' cells (DCs), into HCs by inducing targeted expression of Atoh1 at neonatal and juvenile ages using novel mouse models...
  49. Hayashi T, Ray C, Younkins C, Bermingham McDonogh O. Expression patterns of FGF receptors in the developing mammalian cochlea. Dev Dyn. 2010;239:1019-26 pubmed publisher
    ..We compare the receptor expression pattern to markers of the sensory domain (p27kip1) and the early hair cells (math1).
  50. Noah T, Kazanjian A, Whitsett J, Shroyer N. SAM pointed domain ETS factor (SPDEF) regulates terminal differentiation and maturation of intestinal goblet cells. Exp Cell Res. 2010;316:452-65 pubmed publisher
    ..These data support a model in which Spdef promotes terminal differentiation into goblet cells of a common goblet/Paneth progenitor. ..
  51. Maricich S, Xia A, Mathes E, Wang V, Oghalai J, Fritzsch B, et al. Atoh1-lineal neurons are required for hearing and for the survival of neurons in the spiral ganglion and brainstem accessory auditory nuclei. J Neurosci. 2009;29:11123-33 pubmed publisher
    b>Atoh1 is a basic helix-loop-helix transcription factor necessary for the specification of inner ear hair cells and central auditory system neurons derived from the rhombic lip...
  52. Okubo T, Hogan B. Hyperactive Wnt signaling changes the developmental potential of embryonic lung endoderm. J Biol. 2004;3:11 pubmed
    ..In addition, there was strong ectopic expression of genes such as Cdx1 and Atoh1 that normally regulate gut development and early allocation of cells to intestinal secretory lineages...
  53. Helms A, Abney A, Ben Arie N, Zoghbi H, Johnson J. Autoregulation and multiple enhancers control Math1 expression in the developing nervous system. Development. 2000;127:1185-96 pubmed
    ..b>MATH1, a transcription factor of the bHLH class, is expressed during development of the nervous system in multiple ..
  54. Lee K, Dietrich P, Jessell T. Genetic ablation reveals that the roof plate is essential for dorsal interneuron specification. Nature. 2000;403:734-40 pubmed
    ..These results reveal that the roof plate is essential for specifying multiple classes of neurons in the mammalian central nervous system. ..
  55. Huang W, Tupal S, Huang T, Ward C, Neul J, Klisch T, et al. Atoh1 governs the migration of postmitotic neurons that shape respiratory effectiveness at birth and chemoresponsiveness in adulthood. Neuron. 2012;75:799-809 pubmed publisher
    ..proprioceptive, and arousal functions share a developmental requirement for the bHLH transcription factor Atoh1. Loss of Atoh1 in mice results in respiratory failure and neonatal lethality; however, the neuronal identity and ..
  56. van Es J, De Geest N, van de Born M, Clevers H, Hassan B. Intestinal stem cells lacking the Math1 tumour suppressor are refractory to Notch inhibitors. Nat Commun. 2010;1:18 pubmed publisher
    ..The basic helix-loop-helix transcription factor Math1 is essential for intestinal secretory cell differentiation...
  57. Dubreuil V, Thoby Brisson M, Rallu M, Persson K, Pattyn A, Birchmeier C, et al. Defective respiratory rhythmogenesis and loss of central chemosensitivity in Phox2b mutants targeting retrotrapezoid nucleus neurons. J Neurosci. 2009;29:14836-46 pubmed publisher
    ..RTN) is a group of neurons in the rostral medulla, defined here as Phox2b-, Vglut2-, neurokinin1 receptor-, and Atoh1-expressing cells in the parafacial region, which have been proposed to function both as generators of respiratory ..
  58. Matei V, Pauley S, Kaing S, Rowitch D, Beisel K, Morris K, et al. Smaller inner ear sensory epithelia in Neurog 1 null mice are related to earlier hair cell cycle exit. Dev Dyn. 2005;234:633-50 pubmed
    ..We tested the possible expression of Atoh1 in neurosensory precursors using two Atoh 1-Cre lines...
  59. Lumpkin E, Collisson T, Parab P, Omer Abdalla A, Haeberle H, Chen P, et al. Math1-driven GFP expression in the developing nervous system of transgenic mice. Gene Expr Patterns. 2003;3:389-95 pubmed
    b>Math1 is a bHLH transcription factor expressed in neural progenitor cells in multiple regions of the nervous system...
  60. Ben Arie N, Hassan B, Bermingham N, Malicki D, Armstrong D, Matzuk M, et al. Functional conservation of atonal and Math1 in the CNS and PNS. Development. 2000;127:1039-48 pubmed
    To determine the extent to which atonal and its mouse homolog Math1 exhibit functional conservation, we inserted (beta)-galactosidase (lacZ) into the Math1 locus and analyzed its expression, evaluated consequences of loss of Math1 ..
  61. Wang V, Rose M, Zoghbi H. Math1 expression redefines the rhombic lip derivatives and reveals novel lineages within the brainstem and cerebellum. Neuron. 2005;48:31-43 pubmed
    ..We use beta-galactosidase expressed from the Math1 locus in Math1-heterozygous and Math1-null mice to track RL-derived cells and to evaluate their developmental ..
  62. Fritzsch B, Matei V, Nichols D, Bermingham N, Jones K, Beisel K, et al. Atoh1 null mice show directed afferent fiber growth to undifferentiated ear sensory epithelia followed by incomplete fiber retention. Dev Dyn. 2005;233:570-83 pubmed
    ..b>Atoh1 null mice never fully differentiate hair cells and supporting cells and, therefore, may show aberrations in the ..
  63. Pascual M, Abasolo I, Mingorance Le Meur A, MARTINEZ A, Del Río J, Wright C, et al. Cerebellar GABAergic progenitors adopt an external granule cell-like phenotype in the absence of Ptf1a transcription factor expression. Proc Natl Acad Sci U S A. 2007;104:5193-8 pubmed produce PCs and interneurons, aberrantly migrate to the EGL and express typical markers of these cells, such as Math1, Reelin, and Zic1/2...
  64. Pauley S, Lai E, Fritzsch B. Foxg1 is required for morphogenesis and histogenesis of the mammalian inner ear. Dev Dyn. 2006;235:2470-82 pubmed
    ..Much of the Foxg1 phenotype can be explained by the participation of the protein binding domain in the delta/notch/hes signaling pathway. Additional Foxg1 effects may be mediated by the forkhead DNA binding domain. ..
  65. Pan N, Jahan I, Kersigo J, Kopecky B, Santi P, Johnson S, et al. Conditional deletion of Atoh1 using Pax2-Cre results in viable mice without differentiated cochlear hair cells that have lost most of the organ of Corti. Hear Res. 2011;275:66-80 pubmed publisher
    Atonal homolog1 (Atoh1, formerly Math1) is a crucial bHLH transcription factor for inner ear hair cell differentiation...
  66. Rose M, Ren J, Ahmad K, Chao H, Klisch T, Flora A, et al. Math1 is essential for the development of hindbrain neurons critical for perinatal breathing. Neuron. 2009;64:341-54 pubmed publisher
    Mice lacking the proneural transcription factor Math1 (Atoh1) lack multiple neurons of the proprioceptive and arousal systems and die shortly after birth from an apparent inability to initiate respiration...
  67. Fritzsch B, Dillard M, Lavado A, Harvey N, Jahan I. Canal cristae growth and fiber extension to the outer hair cells of the mouse ear require Prox1 activity. PLoS ONE. 2010;5:e9377 pubmed publisher
    ..These results identify a dual role of Prox1 during inner ear development; growth of the canal cristae and fiber guidance of Type II fibers along supporting cells in the cochlea. ..
  68. Gazit R, Krizhanovsky V, Ben Arie N. Math1 controls cerebellar granule cell differentiation by regulating multiple components of the Notch signaling pathway. Development. 2004;131:903-13 pubmed
    ..b>Math1 is a bHLH transcription activator that is essential for the genesis of CGC...
  69. Maricich S, Wellnitz S, Nelson A, Lesniak D, Gerling G, Lumpkin E, et al. Merkel cells are essential for light-touch responses. Science. 2009;324:1580-2 pubmed publisher
    ..We used Cre-loxP technology to conditionally delete the transcription factor Atoh1 from the body skin and foot pads of mice. Merkel cells are absent from these areas in Atoh1(CKO) animals...
  70. Hayashi T, Cunningham D, Bermingham McDonogh O. Loss of Fgfr3 leads to excess hair cell development in the mouse organ of Corti. Dev Dyn. 2007;236:525-33 pubmed
    ..Thus, in addition to controlling the fate decision between pillar cells and Deiters' cells, we find that Fgfr3 also regulates the width of the sensory epithelium...
  71. Engelkamp D, Rashbass P, Seawright A, van Heyningen V. Role of Pax6 in development of the cerebellar system. Development. 1999;126:3585-96 pubmed
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