Gene Symbol: Myo7a
Description: myosin VIIA
Alias: Hdb, Myo7, USH1B, nmf371, polka, sh-1, sh1, unconventional myosin-VIIa, motor protein, myosin-VIIa, shaker 1
Species: mouse
Products:     Myo7a

Top Publications

  1. 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
    ..These results suggest important roles for Rbpj and notch signaling in multiple aspects of inner ear development including prosensory cell maturation, cellular differentiation and survival. ..
  2. El Amraoui A, Sahly I, Picaud S, Sahel J, Abitbol M, Petit C. Human Usher 1B/mouse shaker-1: the retinal phenotype discrepancy explained by the presence/absence of myosin VIIA in the photoreceptor cells. Hum Mol Genet. 1996;5:1171-8 pubmed
    ..The gene encoding myosin VIIA is responsible for USH1B...
  3. Reiners J, Nagel Wolfrum K, Jürgens K, Märker T, Wolfrum U. Molecular basis of human Usher syndrome: deciphering the meshes of the Usher protein network provides insights into the pathomechanisms of the Usher disease. Exp Eye Res. 2006;83:97-119 pubmed
    ..There are five known USH1 molecules: the molecular motor myosin VIIa (USH1B); the two cell-cell adhesion cadherin proteins, cadherin 23 (USH1D) and protocadherin 15, (USH1F) and ..
  4. Bermingham McDonogh O, Oesterle E, Stone J, Hume C, Huynh H, Hayashi T. Expression of Prox1 during mouse cochlear development. J Comp Neurol. 2006;496:172-86 pubmed
    ..Expression is down regulated in maturing (myosin VIIA immunoreactive) vestibular hair cells and subsequently in the underlying support cell layer by E16.5...
  5. Gibbs D, Kitamoto J, Williams D. Abnormal phagocytosis by retinal pigmented epithelium that lacks myosin VIIa, the Usher syndrome 1B protein. Proc Natl Acad Sci U S A. 2003;100:6481-6 pubmed
    Mutations in the myosin VIIa gene (MYO7A) cause Usher syndrome type 1B (USH1B), a major type of the deaf-blind disorder, Usher syndrome...
  6. Mburu P, Mustapha M, Varela A, Weil D, El Amraoui A, Holme R, et al. Defects in whirlin, a PDZ domain molecule involved in stereocilia elongation, cause deafness in the whirler mouse and families with DFNB31. Nat Genet. 2003;34:421-8 pubmed
    ..Our findings suggest that this novel PDZ domain-containing molecule acts as an organizer of submembranous molecular complexes that control the coordinated actin polymerization and membrane growth of stereocilia. ..
  7. Zine A, Aubert A, Qiu J, Therianos S, Guillemot F, Kageyama R, et al. Hes1 and Hes5 activities are required for the normal development of the hair cells in the mammalian inner ear. J Neurosci. 2001;21:4712-20 pubmed
    ..These data indicate that Hes1 and Hes5 participate together for the control of inner ear hair cell production, likely through the negative regulation of Math1. ..
  8. Gibbs D, Azarian S, Lillo C, Kitamoto J, Klomp A, Steel K, et al. Role of myosin VIIa and Rab27a in the motility and localization of RPE melanosomes. J Cell Sci. 2004;117:6473-83 pubmed
    b>Myosin VIIa functions in the outer retina, and loss of this function causes human blindness in Usher syndrome type 1B (USH1B). In mice with mutant Myo7a, melanosomes in the retinal pigmented epithelium (RPE) are distributed abnormally...
  9. Kiernan A, Cordes R, Kopan R, Gossler A, Gridley T. The Notch ligands DLL1 and JAG2 act synergistically to regulate hair cell development in the mammalian inner ear. Development. 2005;132:4353-62 pubmed
    ..Our results demonstrate that the Notch pathway plays a dual role in regulating cellular differentiation and patterning in the cochlea, acting both through lateral inhibition and the control of cellular proliferation...

More Information


  1. Zou D, Erickson C, Kim E, Jin D, Fritzsch B, Xu P. Eya1 gene dosage critically affects the development of sensory epithelia in the mammalian inner ear. Hum Mol Genet. 2008;17:3340-56 pubmed publisher
    ..These results also provide a molecular mechanism for understanding how hypomorphic levels of EYA1 cause inner-ear defects in humans. ..
  2. Liu X, Walsh J, Mburu P, Kendrick Jones J, Cope M, Steel K, et al. Mutations in the myosin VIIA gene cause non-syndromic recessive deafness. Nat Genet. 1997;16:188-90 pubmed
    ..Recently, it has been shown that a gene encoding an unconventional myosin, myosin VIIA, underlies the mouse recessive deafness mutation, shaker-1 (ref. 5) as well as Usher syndrome type 1b...
  3. Lefevre G, Michel V, Weil D, Lepelletier L, Bizard E, Wolfrum U, et al. A core cochlear phenotype in USH1 mouse mutants implicates fibrous links of the hair bundle in its cohesion, orientation and differential growth. Development. 2008;135:1427-37 pubmed publisher
    ..Mutations in genes encoding myosin VIIa, harmonin, cadherin 23, protocadherin 15 or sans cause Usher syndrome type I (USH1, characterized by congenital ..
  4. 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
  5. Jahan I, Kersigo J, Pan N, Fritzsch B. Neurod1 regulates survival and formation of connections in mouse ear and brain. Cell Tissue Res. 2010;341:95-110 pubmed publisher
  6. Wakaoka T, Motohashi T, Hayashi H, Kuze B, Aoki M, Mizuta K, et al. Tracing Sox10-expressing cells elucidates the dynamic development of the mouse inner ear. Hear Res. 2013;302:17-25 pubmed publisher
    ..Further analyzing the Sox10-IRES-Venus mice would provide important information to better understand the development of the inner ear...
  7. Delprat B, Michel V, Goodyear R, Yamasaki Y, Michalski N, El Amraoui A, et al. Myosin XVa and whirlin, two deafness gene products required for hair bundle growth, are located at the stereocilia tips and interact directly. Hum Mol Genet. 2005;14:401-10 pubmed
    ..Notably, whirlin also interacts with myosin VIIa that is present along the entire length of the stereocilia...
  8. Deans M, Antic D, Suyama K, Scott M, Axelrod J, Goodrich L. Asymmetric distribution of prickle-like 2 reveals an early underlying polarization of vestibular sensory epithelia in the inner ear. J Neurosci. 2007;27:3139-47 pubmed
  9. Gibson F, Walsh J, Mburu P, Varela A, Brown K, Antonio M, et al. A type VII myosin encoded by the mouse deafness gene shaker-1. Nature. 1995;374:62-4 pubmed
    ..Here we report the identification of one such gene, the mouse shaker-1 (sh1) gene...
  10. 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. ..
  11. Prosser H, Rzadzinska A, Steel K, Bradley A. Mosaic complementation demonstrates a regulatory role for myosin VIIa in actin dynamics of stereocilia. Mol Cell Biol. 2008;28:1702-12 pubmed
    We have developed a bacterial artificial chromosome transgenesis approach that allowed the expression of myosin VIIa from the mouse X chromosome...
  12. 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
    ..Our findings demonstrate that direct and cooperative interactions between the Sox2, Six1, and Eya1 proteins coordinate Atoh1 expression to specify hair cell fate. ..
  13. Mantela J, Jiang Z, Ylikoski J, Fritzsch B, Zacksenhaus E, Pirvola U. The retinoblastoma gene pathway regulates the postmitotic state of hair cells of the mouse inner ear. Development. 2005;132:2377-88 pubmed
    ..Our findings demonstrate that the pRb pathway is required for hair cell quiescence and that manipulation of the cell cycle machinery disrupts the coordinated development within the inner ear sensory epithelia. ..
  14. Peng Y, Zallocchi M, Wang W, Delimont D, Cosgrove D. Moderate light-induced degeneration of rod photoreceptors with delayed transducin translocation in shaker1 mice. Invest Ophthalmol Vis Sci. 2011;52:6421-7 pubmed publisher
    ..Mutations in the myosin VIIa gene (MYO7A) cause a common and severe subtype of Usher syndrome (USH1B). Shaker1 mice have mutant MYO7A...
  15. Lopes V, Gibbs D, Libby R, Aleman T, Welch D, Lillo C, et al. The Usher 1B protein, MYO7A, is required for normal localization and function of the visual retinoid cycle enzyme, RPE65. Hum Mol Genet. 2011;20:2560-70 pubmed publisher
    Mutations in the MYO7A gene cause a deaf-blindness disorder, known as Usher syndrome 1B...
  16. Liu X, Udovichenko I, Brown S, Steel K, Williams D. Myosin VIIa participates in opsin transport through the photoreceptor cilium. J Neurosci. 1999;19:6267-74 pubmed
    Two types of Usher syndrome, a blindness-deafness disorder, result from mutations in the myosin VIIa gene. As for most other unconventional myosins, little is known about the function or functions of myosin VIIa...
  17. Saw D, Steel K, Brown S. Shaker mice and a peek into the House of Usher. Exp Anim. 1997;46:1-9 pubmed
  18. 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. ..
  19. Goodyear R, Legan P, Wright M, Marcotti W, Oganesian A, Coats S, et al. A receptor-like inositol lipid phosphatase is required for the maturation of developing cochlear hair bundles. J Neurosci. 2003;23:9208-19 pubmed
    ..These results reveal that Ptprq is required for formation of the shaft connectors of the hair bundle, the normal maturation of cochlear hair bundles, and the long-term survival of high-frequency auditory hair cells. ..
  20. Adato A, Michel V, Kikkawa Y, Reiners J, Alagramam K, Weil D, et al. Interactions in the network of Usher syndrome type 1 proteins. Hum Mol Genet. 2005;14:347-56 pubmed
    Defects in myosin VIIa, harmonin (a PDZ domain protein), cadherin 23, protocadherin 15 and sans (a putative scaffolding protein), underlie five forms of Usher syndrome type I (USH1)...
  21. Freyer L, Aggarwal V, Morrow B. Dual embryonic origin of the mammalian otic vesicle forming the inner ear. Development. 2011;138:5403-14 pubmed publisher
    ..This study defines a dual cellular origin of the inner ear from sensory placode ectoderm and NECs, and changes the current paradigm of inner ear neurosensory development. ..
  22. 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
    ..Results suggest that hair cell miRNAs subdue cochlear gradient gene expression and are required for hair cell maintenance and survival. ..
  23. Hashimoto T, Gibbs D, Lillo C, Azarian S, Legacki E, Zhang X, et al. Lentiviral gene replacement therapy of retinas in a mouse model for Usher syndrome type 1B. Gene Ther. 2007;14:584-94 pubmed
    ..Mutations in the myosin VIIa gene (MYO7A) cause a major subtype of Usher syndrome, type 1B...
  24. 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. ..
  25. 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
    ..The remaining cells express hair cell marker Myo7a and attract nerve fibers, but do not differentiate normal stereocilia bundles...
  26. 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. ..
  27. Dong Y, Sui L, Yamaguchi F, Kamitori K, Hirata Y, Hossain M, et al. Phosphatase and tensin homolog deleted on chromosome 10 regulates sensory cell proliferation and differentiation of hair bundles in the mammalian cochlea. Neuroscience. 2010;170:1304-13 pubmed publisher
  28. Lee Y, Liu F, Segil N. A morphogenetic wave of p27Kip1 transcription directs cell cycle exit during organ of Corti development. Development. 2006;133:2817-26 pubmed
    ..This report establishes a link between the spatial and temporal pattern of p27(Kip1) transcription and the control of cell number during sensory organ morphogenesis. ..
  29. Holme R, Steel K. Stereocilia defects in waltzer (Cdh23), shaker1 (Myo7a) and double waltzer/shaker1 mutant mice. Hear Res. 2002;169:13-23 pubmed
    Mutations in myosin VIIa (Myo7a) and cadherin 23 (Cdh23) cause deafness in shaker1 (sh1) and waltzer (v) mouse mutants respectively...
  30. 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
    ..Through this and other feedback, Neurod1 suppresses alternate fates of neurons to differentiate as hair cells and regulates hair cell subtypes. ..
  31. Hartman B, Reh T, Bermingham McDonogh O. Notch signaling specifies prosensory domains via lateral induction in the developing mammalian inner ear. Proc Natl Acad Sci U S A. 2010;107:15792-7 pubmed publisher
    ..These results support a model where activation of Notch and propagation through lateral induction promote prosensory character in specific regions of the developing otocyst. ..
  32. Gibbs D, Cideciyan A, Jacobson S, Williams D. Retinal pigment epithelium defects in humans and mice with mutations in MYO7A: imaging melanosome-specific autofluorescence. Invest Ophthalmol Vis Sci. 2009;50:4386-93 pubmed publisher
    ..under development for use in the most common genetic variant of USH1, USH1B, which is caused by mutations in the MYO7A gene...
  33. Kersigo J, D Angelo A, Gray B, Soukup G, Fritzsch B. The role of sensory organs and the forebrain for the development of the craniofacial shape as revealed by Foxg1-cre-mediated microRNA loss. Genesis. 2011;49:326-41 pubmed publisher
  34. Togashi H, Kominami K, Waseda M, Komura H, Miyoshi J, Takeichi M, et al. Nectins establish a checkerboard-like cellular pattern in the auditory epithelium. Science. 2011;333:1144-7 pubmed publisher
    ..When cells expressing either nectin-1 or -3 were cocultured, they arranged themselves into a mosaic pattern. Thus, nectin-1 and -3 promote the formation of the checkerboard-like pattern of the auditory epithelia. ..
  35. 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. ..
  36. Duncan J, Fritzsch B. Continued expression of GATA3 is necessary for cochlear neurosensory development. PLoS ONE. 2013;8:e62046 pubmed publisher
    ..We suggest that Gata3 may act in combination with Eya1, Six1, and Sox2 in cochlear prosensory gene signaling...
  37. Weil D, Blanchard S, Kaplan J, Guilford P, Gibson F, Walsh J, et al. Defective myosin VIIA gene responsible for Usher syndrome type 1B. Nature. 1995;374:60-1 pubmed
    ..Here we present evidence that a gene encoding myosin VIIA is responsible for USH1B...
  38. Schwander M, Lopes V, Sczaniecka A, Gibbs D, Lillo C, Delano D, et al. A novel allele of myosin VIIa reveals a critical function for the C-terminal FERM domain for melanosome transport in retinal pigment epithelial cells. J Neurosci. 2009;29:15810-8 pubmed publisher
    Mutations in the head and tail domains of the motor protein myosin VIIA (MYO7A) cause deaf-blindness (Usher syndrome type 1B, USH1B) and nonsyndromic deafness (DFNB2, DFNA11)...
  39. Li S, Price S, Cahill H, Ryugo D, Shen M, Xiang M. Hearing loss caused by progressive degeneration of cochlear hair cells in mice deficient for the Barhl1 homeobox gene. Development. 2002;129:3523-32 pubmed
    ..Our data together indicate an essential role for Barhl1 in the long-term maintenance of cochlear hair cells, but not in the determination or differentiation of these cells. ..
  40. Mburu P, Liu X, Walsh J, Saw D, Cope M, Gibson F, et al. Mutation analysis of the mouse myosin VIIA deafness gene. Genes Funct. 1997;1:191-203 pubmed
    The shaker-1 (Myo7a) mouse deafness locus is encoded by an unconventional myosin gene: myosin VIIA [Gibson, Walsh, Mburu, Varela, Brown, Antonio, Biesel, Steel and Brown (1995) Nature (London) 374, 62-64]...
  41. Belyantseva I, Boger E, Naz S, Frolenkov G, Sellers J, Ahmed Z, et al. Myosin-XVa is required for tip localization of whirlin and differential elongation of hair-cell stereocilia. Nat Cell Biol. 2005;7:148-56 pubmed
    ..We show that myosin-XVa is a motor protein that, in vivo, interacts with the third PDZ domain of whirlin through its carboxy-terminal PDZ-ligand...
  42. Chai R, Xia A, Wang T, Jan T, Hayashi T, Bermingham McDonogh O, et al. Dynamic expression of Lgr5, a Wnt target gene, in the developing and mature mouse cochlea. J Assoc Res Otolaryngol. 2011;12:455-69 pubmed publisher
    ..Their differential expression among cell populations highlights the dynamic but complex distribution of Wnt-activated cells in and around the embryonic and postnatal cochlea...
  43. Senften M, Schwander M, Kazmierczak P, Lillo C, Shin J, Hasson T, et al. Physical and functional interaction between protocadherin 15 and myosin VIIa in mechanosensory hair cells. J Neurosci. 2006;26:2060-71 pubmed
    ..gene products that have been associated with deafness and hair bundle defects, protocadherin 15 (PCDH15) and myosin VIIa (MYO7A), into a common pathway...
  44. Udovichenko I, Gibbs D, Williams D. Actin-based motor properties of native myosin VIIa. J Cell Sci. 2002;115:445-50 pubmed
    b>Myosin VIIa has critical roles in the inner ear and the retina. To help understand how this protein functions, native myosin VIIa was tested for mechanoenzymatic properties...
  45. Boëda B, El Amraoui A, Bahloul A, Goodyear R, Daviet L, Blanchard S, et al. Myosin VIIa, harmonin and cadherin 23, three Usher I gene products that cooperate to shape the sensory hair cell bundle. EMBO J. 2002;21:6689-99 pubmed
    Deaf-blindness in three distinct genetic forms of Usher type I syndrome (USH1) is caused by defects in myosin VIIa, harmonin and cadherin 23...
  46. Kopecky B, Santi P, Johnson S, Schmitz H, Fritzsch B. Conditional deletion of N-Myc disrupts neurosensory and non-sensory development of the ear. Dev Dyn. 2011;240:1373-90 pubmed publisher
    ..These findings are put in the context of the possible functional relationship of N-Myc with a number of other cell proliferative and fate determining genes during ear development. ..
  47. Libby R, Steel K. Electroretinographic anomalies in mice with mutations in Myo7a, the gene involved in human Usher syndrome type 1B. Invest Ophthalmol Vis Sci. 2001;42:770-8 pubmed
    In humans, mutations in the gene encoding myosin VIIa can cause Usher syndrome type 1b (USH1B), a disease characterized by deafness and retinitis pigmentosa...
  48. Chen P, Segil N. p27(Kip1) links cell proliferation to morphogenesis in the developing organ of Corti. Development. 1999;126:1581-90 pubmed
    ..Homozygous mutant mice are severely hearing impaired. Thus, p27(Kip1) provides a link between developmental control of cell proliferation and the morphological development of the inner ear. ..
  49. Liu X, Ondek B, Williams D. Mutant myosin VIIa causes defective melanosome distribution in the RPE of shaker-1 mice. Nat Genet. 1998;19:117-8 pubmed
  50. Kirjavainen A, Sulg M, Heyd F, Alitalo K, Yla Herttuala S, Moroy T, et al. Prox1 interacts with Atoh1 and Gfi1, and regulates cellular differentiation in the inner ear sensory epithelia. Dev Biol. 2008;322:33-45 pubmed publisher
    ..The data point to the tight regulation of phenotypic characteristics of hair cells and supporting cells. ..
  51. Kros C, Marcotti W, van Netten S, Self T, Libby R, Brown S, et al. Reduced climbing and increased slipping adaptation in cochlear hair cells of mice with Myo7a mutations. Nat Neurosci. 2002;5:41-7 pubmed
    ..Transducer currents also adapt more strongly than normal to excitatory stimuli. We conclude that myosin VIIA participates in anchoring and holding membrane-bound elements to the actin core of the stereocilium...
  52. Xiang M, Gao W, Hasson T, Shin J. Requirement for Brn-3c in maturation and survival, but not in fate determination of inner ear hair cells. Development. 1998;125:3935-46 pubmed
    ..These data indicate a crucial role for Brn-3c in maturation, survival and migration of hair cells, but not in proliferation or commitment of hair cell progenitors. ..
  53. Hasson T, Heintzelman M, Santos Sacchi J, Corey D, Mooseker M. Expression in cochlea and retina of myosin VIIa, the gene product defective in Usher syndrome type 1B. Proc Natl Acad Sci U S A. 1995;92:9815-9 pubmed
    b>Myosin VIIa is a newly identified member of the myosin superfamily of actin-based motors. Recently, the myosin VIIa gene was identified as the gene defective in shaker-1, a recessive deafness in mice [Gibson, F., Walsh, J., Mburu, P...