1. LewisAK, FrantzGD, CarpenterDA, de SauvageFJ, GaoWQ (1998) Distinct expression patterns of notch family receptors and ligands during development of the mammalian inner ear. Mech Dev 78: 159–163.
2. MurataJ, TokunagaA, OkanoH, KuboT (2006) Mapping of notch activation during cochlear development in mice: implications for determination of prosensory domain and cell fate diversification. J Comp Neurol 497: 502–518.
3. MorrisonA, HodgettsC, GosslerA, Hrabe de AngelisM, LewisJ (1999) Expression of Delta1 and Serrate1 (Jagged1) in the mouse inner ear. Mech Dev 84: 169–172.
4. HartmanBH, HayashiT, NelsonBR, Bermingham-McDonoghO, RehTA (2007) Dll3 is expressed in developing hair cells in the mammalian cochlea. Dev Dyn 236: 2875–2883.
5. KiernanAE, CordesR, KopanR, GosslerA, GridleyT (2005) The Notch ligands DLL1 and JAG2 act synergistically to regulate hair cell development in the mammalian inner ear. Development 132: 4353–4362.
6. BrookerR, HozumiK, LewisJ (2006) Notch ligands with contrasting functions: Jagged1 and Delta1 in the mouse inner ear. Development 133: 1277–1286.
7. LanfordPJ, LanY, JiangR, LindsellC, WeinmasterG, et al. (1999) Notch signalling pathway mediates hair cell development in mammalian cochlea. Nat Genet 21: 289–292.
8. ZhangN, MartinGV, KelleyMW, GridleyT (2000) A mutation in the Lunatic fringe gene suppresses the effects of a Jagged2 mutation on inner hair cell development in the cochlea. Curr Biol 10: 659–662.
9. DoetzlhoferA, BaschML, OhyamaT, GesslerM, GrovesAK, et al. (2009) Hey2 regulation by FGF provides a Notch-independent mechanism for maintaining pillar cell fate in the organ of Corti. Dev Cell 16: 58–69.
10. TateyaT, ImayoshiI, TateyaI, ItoJ, KageyamaR (2011) Cooperative functions of Hes/Hey genes in auditory hair cell and supporting cell development. Dev Biol 352: 329–340.
11. LiS, MarkS, Radde-GallwitzK, SchlisnerR, ChinMT, et al. (2008) Hey2 functions in parallel with Hes1 and Hes5 for mammalian auditory sensory organ development. BMC Dev Biol 8: 20.
12. OhyamaT, BaschML, MishinaY, LyonsKM, SegilN, et al. (2010) BMP signaling is necessary for patterning the sensory and nonsensory regions of the developing mammalian cochlea. J Neurosci 30: 15044–15051.
13. KiernanAE, XuJ, GridleyT (2006) The Notch ligand JAG1 is required for sensory progenitor development in the mammalian inner ear. PLoS Genet 2: e4.
14. KiernanAE, AhituvN, FuchsH, BallingR, AvrahamKB, et al. (2001) The Notch ligand Jagged1 is required for inner ear sensory development. Proc Natl Acad Sci U S A 98: 3873–3878.
15. TsaiH, HardistyRE, RhodesC, KiernanAE, RobyP, et al. (2001) The mouse slalom mutant demonstrates a role for Jagged1 in neuroepithelial patterning in the organ of Corti. Hum Mol Genet 10: 507–512.
16. GreenMM (1959) Spatial and functional properties of pseudo-alleles at the white locus in Drosophila melanogaster. Heredity 303–315.
17. TwyffelsL, GueydanC, KruysV (2011) Shuttling SR proteins: more than splicing factors. FEBS J 278: 3246–3255.
18. ZacharZ, ChouTB, BinghamPM (1987) Evidence that a regulatory gene autoregulates splicing of its transcript. EMBO J 6: 4105–4111.
19. ZacharZ, ChouTB, KramerJ, MimsIP, BinghamPM (1994) Analysis of autoregulation at the level of pre-mRNA splicing of the suppressor-of-white-apricot gene in Drosophila. Genetics 137: 139–150.
20. RutledgeBJ, MortinMA, SchwarzE, Thierry-MiegD, MeselsonM (1988) Genetic interactions of modifier genes and modifiable alleles in Drosophila melanogaster. Genetics 119: 391–397.
21. SarkissianM, WinneA, LafyatisR (1996) The mammalian homolog of suppressor-of-white-apricot regulates alternative mRNA splicing of CD45 exon 4 and fibronectin IIICS. J Biol Chem 271: 31106–31114.
22. DenhezF, LafyatisR (1994) Conservation of regulated alternative splicing and identification of functional domains in vertebrate homologs to the Drosophila splicing regulator, suppressor-of-white-apricot. J Biol Chem 269: 16170–16179.
23. LemaireR, WinneA, SarkissianM, LafyatisR (1999) SF2 and SRp55 regulation of CD45 exon 4 skipping during T cell activation. Eur J Immunol 29: 823–837.
24. MountSM, GreenMM, RubinGM (1988) Partial revertants of the transposable element-associated suppressible allele white-apricot in Drosophila melanogaster: structures and responsiveness to genetic modifiers. Genetics 118: 221–234.
25. Overbeek PA (2002) Factors affecting transgenic animal production; Pinkert C, editor. New York, NY: Elsevier Science.
26. Hardisty-HughesRE, ParkerA, BrownSD (2010) A hearing and vestibular phenotyping pipeline to identify mouse mutants with hearing impairment. Nat Protoc 5: 177–190.
27. SalviRJ, DingD, WangJ, JiangHY (2000) A review of the effects of selective inner hair cell lesions on distortion product otoacoustic emissions, cochlear function and auditory evoked potentials. Noise Health 2: 9–26.
28. PaylorR, CrawleyJN (1997) Inbred strain differences in prepulse inhibition of the mouse startle response. Psychopharmacology (Berl) 132: 169–180.
29. Bermingham-McDonoghO, OesterleEC, StoneJS, HumeCR, HuynhHM, et al. (2006) Expression of Prox1 during mouse cochlear development. J Comp Neurol 496: 172–186.
30. MorsliH, ChooD, RyanA, JohnsonR, WuDK (1998) Development of the mouse inner ear and origin of its sensory organs. J Neurosci 18: 3327–3335.
31. MeyersJR, MacDonaldRB, DugganA, LenziD, StandaertDG, et al. (2003) Lighting up the senses: FM1-43 loading of sensory cells through nonselective ion channels. J Neurosci 23: 4054–4065.
32. PanW, JinY, StangerB, KiernanAE (2010) Notch signaling is required for the generation of hair cells and supporting cells in the mammalian inner ear. Proc Natl Acad Sci U S A 107: 15798–15803.
33. KiernanAE, LiR, HawesNL, ChurchillGA, GridleyT (2007) Genetic background modifies inner ear and eye phenotypes of jag1 heterozygous mice. Genetics 177: 307–311.
34. ChangW, LinZ, KulessaH, HebertJ, HoganBL, et al. (2008) Bmp4 is essential for the formation of the vestibular apparatus that detects angular head movements. PLoS Genet 4: e1000050.
35. KoutelouE, SatoS, Tomomori-SatoC, FlorensL, SwansonSK, et al. (2008) Neuralized-like 1 (Neurl1) targeted to the plasma membrane by N-myristoylation regulates the Notch ligand Jagged1. J Biol Chem 283: 3846–3853.
36. McGillMA, DhoSE, WeinmasterG, McGladeCJ (2009) Numb regulates post-endocytic trafficking and degradation of Notch1. J Biol Chem 284: 26427–26438.
37. McGillMA, McGladeCJ (2003) Mammalian numb proteins promote Notch1 receptor ubiquitination and degradation of the Notch1 intracellular domain. J Biol Chem 278: 23196–23203.
38. GaoZ, ChiFL, HuangYB, YangJM, CongN, et al. (2011) Expression of Numb and Numb-like in the development of mammalian auditory sensory epithelium. Neuroreport 22: 49–54.
39. NamY, SlizP, SongL, AsterJC, BlacklowSC (2006) Structural basis for cooperativity in recruitment of MAML coactivators to Notch transcription complexes. Cell 124: 973–983.
40. FukamiM, WadaY, OkadaM, KatoF, KatsumataN, et al. (2008) Mastermind-like domain-containing 1 (MAMLD1 or CXorf6) transactivates the Hes3 promoter, augments testosterone production, and contains the SF1 target sequence. J Biol Chem 283: 5525–5532.
41. WuL, SunT, KobayashiK, GaoP, GriffinJD (2002) Identification of a family of mastermind-like transcriptional coactivators for mammalian notch receptors. Mol Cell Biol 22: 7688–7700.
42. PirrottaV, BrocklC (1984) Transcription of the Drosophila white locus and some of its mutants. EMBO J 3: 563–568.
43. LevisR, O'HareK, RubinGM (1984) Effects of transposable element insertions on RNA encoded by the white gene of Drosophila. Cell 38: 471–481.
44. ZacharZ, DavisonD, GarzaD, BinghamPM (1985) A detailed developmental and structural study of the transcriptional effects of insertion of the Copia transposon into the white locus of Drosophila melanogaster. Genetics 111: 495–515.
45. BlantonSH, LiangCY, CaiMW, PandyaA, DuLL, et al. (2002) A novel locus for autosomal dominant non-syndromic deafness (DFNA41) maps to chromosome 12q24-qter. J Med Genet 39: 567–570.
46. ChenQ, ChuH, WuX, CuiY, ChenJ, et al. (2011) The expression of plasma membrane Ca(2+)-ATPase isoform 2 and its splice variants at sites A and C in the neonatal rat cochlea. Int J Pediatr Otorhinolaryngol 75: 196–201.
47. BeiselKW, Rocha-SanchezSM, MorrisKA, NieL, FengF, et al. (2005) Differential expression of KCNQ4 in inner hair cells and sensory neurons is the basis of progressive high-frequency hearing loss. J Neurosci 25: 9285–9293.
48. Rocha-SanchezSM, MorrisKA, KacharB, NicholsD, FritzschB, et al. (2007) Developmental expression of Kcnq4 in vestibular neurons and neurosensory epithelia. Brain Res 1139: 117–125.
49. ShenY, YuD, HielH, LiaoP, YueDT, et al. (2006) Alternative splicing of the Ca(v)1.3 channel IQ domain, a molecular switch for Ca2+-dependent inactivation within auditory hair cells. J Neurosci 26: 10690–10699.
50. RamanathanK, MichaelTH, JiangGJ, HielH, FuchsPA (1999) A molecular mechanism for electrical tuning of cochlear hair cells. Science 283: 215–217.
51. LangerP, GrunderS, RuschA (2003) Expression of Ca2+-activated BK channel mRNA and its splice variants in the rat cochlea. J Comp Neurol 455: 198–209.
52. SakaiY, HarveyM, SokolowskiB (2011) Identification and quantification of full-length BK channel variants in the developing mouse cochlea. J Neurosci Res 89: 1747–1760.
53. HousleyGD, KanjhanR, RaybouldNP, GreenwoodD, SalihSG, et al. (1999) Expression of the P2X(2) receptor subunit of the ATP-gated ion channel in the cochlea: implications for sound transduction and auditory neurotransmission. J Neurosci 19: 8377–8388.
54. HillJK, WilliamsDE, LeMasurierM, DumontRA, StrehlerEE, et al. (2006) Splice-site A choice targets plasma-membrane Ca2+-ATPase isoform 2 to hair bundles. J Neurosci 26: 6172–6180.
55. GratiM, AggarwalN, StrehlerEE, WentholdRJ (2006) Molecular determinants for differential membrane trafficking of PMCA1 and PMCA2 in mammalian hair cells. J Cell Sci 119: 2995–3007.
56. FicarellaR, Di LevaF, BortolozziM, OrtolanoS, DonaudyF, et al. (2007) A functional study of plasma-membrane calcium-pump isoform 2 mutants causing digenic deafness. Proc Natl Acad Sci U S A 104: 1516–1521.
57. XuT, NieL, ZhangY, MoJ, FengW, et al. (2007) Roles of alternative splicing in the functional properties of inner ear-specific KCNQ4 channels. J Biol Chem 282: 23899–23909.
58. ChenC, ParkerMS, BarnesAP, DeiningerP, BobbinRP (2000) Functional expression of three P2X(2) receptor splice variants from guinea pig cochlea. J Neurophysiol 83: 1502–1509.
59. BrandleU, SpielmannsP, OsterothR, SimJ, SurprenantA, et al. (1997) Desensitization of the P2X(2) receptor controlled by alternative splicing. FEBS Lett 404: 294–298.
60. LuikartBW, NefS, ShipmanT, ParadaLF (2003) In vivo role of truncated trkb receptors during sensory ganglion neurogenesis. Neuroscience 117: 847–858.
61. NakanoY, JahanI, BondeG, SunX, HildebrandMS, et al. (2012) A mutation in the Srrm4 gene causes alternative splicing defects and deafness in the Bronx waltzer mouse. PLoS Genet 8: e1002966.
62. WebbSW, GrilletN, AndradeLR, XiongW, SwarthoutL, et al. (2011) Regulation of PCDH15 function in mechanosensory hair cells by alternative splicing of the cytoplasmic domain. Development 138: 1607–1617.
63. OuyangXM, XiaXJ, VerpyE, DuLL, PandyaA, et al. (2002) Mutations in the alternatively spliced exons of USH1C cause non-syndromic recessive deafness. Hum Genet 111: 26–30.
64. WhitlonDS, GabelC, ZhangX (1996) Cochlear inner hair cells exist transiently in the fetal Bronx Waltzer (bv/bv) mouse. J Comp Neurol 364: 515–522.
65. KansakuA, HirabayashiS, MoriH, FujiwaraN, KawataA, et al. (2006) Ligand-of-Numb protein X is an endocytic scaffold for junctional adhesion molecule 4. Oncogene 25: 5071–5084.
66. DavisRJ, HardingM, MoayediY, MardonG (2008) Mouse Dach1 and Dach2 are redundantly required for Mullerian duct development. Genesis 46: 205–213.
67. HenriqueD, AdamJ, MyatA, ChitnisA, LewisJ, et al. (1995) Expression of a Delta homologue in prospective neurons in the chick. Nature 375: 787–790.
68. SternCD (1998) Detection of multiple gene products simultaneously by in situ hybridization and immunohistochemistry in whole mounts of avian embryos. Curr Top Dev Biol 36: 223–243.
69. KiernanAE (2006) The paintfill method as a tool for analyzing the three-dimensional structure of the inner ear. Brain Res 1091: 270–276.
70. BaschML, OhyamaT, SegilN, GrovesAK (2011) Canonical Notch signaling is not necessary for prosensory induction in the mouse cochlea: insights from a conditional mutant of RBPjkappa. J Neurosci 31: 8046–8058.
71. XiaA, GaoSS, YuanT, OsbornA, BressA, et al. (2010) Deficient forward transduction and enhanced reverse transduction in the alpha tectorin C1509G human hearing loss mutation. Dis Model Mech 3: 209–223.