메뉴 건너뛰기




Volumn 53, Issue 8, 2016, Pages 5679-5691

SMAD4 Defect Causes Auditory Neuropathy Via Specialized Disruption of Cochlear Ribbon Synapses in Mice

Author keywords

Auditory neuropathy; Cochlea; Deafness; Retina; Ribbon synapse; SMAD4

Indexed keywords

SMAD4 PROTEIN; SMAD4 PROTEIN, MOUSE;

EID: 84944937444     PISSN: 08937648     EISSN: 15591182     Source Type: Journal    
DOI: 10.1007/s12035-015-9454-1     Document Type: Article
Times cited : (10)

References (42)
  • 2
    • 75149151905 scopus 로고    scopus 로고
    • Multi-site diagnosis and management of 260 patients with auditory neuropathy/dys-synchrony (auditory neuropathy spectrum disorder)
    • PID: 20053155
    • Berlin CI, Hood LJ, Morlet T, Wilensky D, Li L, Mattingly KR, Taylor-Jeanfreau J, Keats BJ et al (2010) Multi-site diagnosis and management of 260 patients with auditory neuropathy/dys-synchrony (auditory neuropathy spectrum disorder). Int J Audiol 49(1):30–43
    • (2010) Int J Audiol , vol.49 , Issue.1 , pp. 30-43
    • Berlin, C.I.1    Hood, L.J.2    Morlet, T.3    Wilensky, D.4    Li, L.5    Mattingly, K.R.6    Taylor-Jeanfreau, J.7    Keats, B.J.8
  • 3
    • 84881374344 scopus 로고    scopus 로고
    • Review of hair cell synapse defects in sensorineural hearing impairment
    • PID: 23628789
    • Moser T, Predoehl F, Starr A (2013) Review of hair cell synapse defects in sensorineural hearing impairment. Otol Neurotol 34(6):995–1004
    • (2013) Otol Neurotol , vol.34 , Issue.6 , pp. 995-1004
    • Moser, T.1    Predoehl, F.2    Starr, A.3
  • 4
    • 0037238597 scopus 로고    scopus 로고
    • Non-syndromic recessive auditory neuropathy is the result of mutations in the otoferlin (OTOF) gene
    • COI: 1:CAS:528:DC%2BD3sXht1ymtrk%3D, PID: 12525542
    • Varga R, Kelley PM, Keats BJ, Starr A, Leal SM, Cohn E, Kimberling WJ (2003) Non-syndromic recessive auditory neuropathy is the result of mutations in the otoferlin (OTOF) gene. J Med Genet 40(1):45–50
    • (2003) J Med Genet , vol.40 , Issue.1 , pp. 45-50
    • Varga, R.1    Kelley, P.M.2    Keats, B.J.3    Starr, A.4    Leal, S.M.5    Cohn, E.6    Kimberling, W.J.7
  • 5
    • 33749994043 scopus 로고    scopus 로고
    • Otoferlin, defective in a human deafness form, is essential for exocytosis at the auditory ribbon synapse
    • COI: 1:CAS:528:DC%2BD28XhtFOkt73N, PID: 17055430
    • Roux I, Safieddine S, Nouvian R, Grati M, Simmler MC, Bahloul A, Perfettini I, Le Gall M et al (2006) Otoferlin, defective in a human deafness form, is essential for exocytosis at the auditory ribbon synapse. Cell 127(2):277–289
    • (2006) Cell , vol.127 , Issue.2 , pp. 277-289
    • Roux, I.1    Safieddine, S.2    Nouvian, R.3    Grati, M.4    Simmler, M.C.5    Bahloul, A.6    Perfettini, I.7    Le Gall, M.8
  • 6
    • 48349111572 scopus 로고    scopus 로고
    • Impairment of SLC17A8 encoding vesicular glutamate transporter-3, VGLUT3, underlies nonsyndromic deafness DFNA25 and inner hair cell dysfunction in null mice
    • COI: 1:CAS:528:DC%2BD1cXpvVOhsL4%3D, PID: 18674745
    • Ruel J, Emery S, Nouvian R, Bersot T, Amilhon B, Van Rybroek JM, Rebillard G, Lenoir M et al (2008) Impairment of SLC17A8 encoding vesicular glutamate transporter-3, VGLUT3, underlies nonsyndromic deafness DFNA25 and inner hair cell dysfunction in null mice. Am J Hum Genet 83(2):278–292
    • (2008) Am J Hum Genet , vol.83 , Issue.2 , pp. 278-292
    • Ruel, J.1    Emery, S.2    Nouvian, R.3    Bersot, T.4    Amilhon, B.5    Van Rybroek, J.M.6    Rebillard, G.7    Lenoir, M.8
  • 7
    • 77955787251 scopus 로고    scopus 로고
    • Increased activity of diaphanous homolog 3 (DIAPH3)/diaphanous causes hearing defects in humans with auditory neuropathy and in Drosophila
    • COI: 1:CAS:528:DC%2BC3cXht1GrurnL, PID: 20624953
    • Schoen CJ, Emery SB, Thorne MC, Ammana HR, Sliwerska E, Arnett J, Hortsch M, Hannan F et al (2010) Increased activity of diaphanous homolog 3 (DIAPH3)/diaphanous causes hearing defects in humans with auditory neuropathy and in Drosophila. Proc Natl Acad Sci U S A 107(30):13396–13401
    • (2010) Proc Natl Acad Sci U S A , vol.107 , Issue.30 , pp. 13396-13401
    • Schoen, C.J.1    Emery, S.B.2    Thorne, M.C.3    Ammana, H.R.4    Sliwerska, E.5    Arnett, J.6    Hortsch, M.7    Hannan, F.8
  • 8
    • 0032947634 scopus 로고    scopus 로고
    • A mutation in OTOF, encoding otoferlin, a FER-1-like protein, causes DFNB9, a nonsyndromic form of deafness
    • COI: 1:CAS:528:DyaK1MXitFKrs70%3D, PID: 10192385
    • Yasunaga S, Grati M, Cohen-Salmon M, El-Amraoui A, Mustapha M, Salem N, El-Zir E, Loiselet J et al (1999) A mutation in OTOF, encoding otoferlin, a FER-1-like protein, causes DFNB9, a nonsyndromic form of deafness. Nat Genet 21(4):363–369
    • (1999) Nat Genet , vol.21 , Issue.4 , pp. 363-369
    • Yasunaga, S.1    Grati, M.2    Cohen-Salmon, M.3    El-Amraoui, A.4    Mustapha, M.5    Salem, N.6    El-Zir, E.7    Loiselet, J.8
  • 9
    • 67650831266 scopus 로고    scopus 로고
    • Chondrocyte-specific Smad4 gene conditional knockout results in hearing loss and inner ear malformation in mice
    • COI: 1:CAS:528:DC%2BD1MXhtVKrsbnM
    • Yang SM, Hou ZH, Yang G, Zhang JS, Hu YY, Sun JH, Guo WW, He D et al (2009) Chondrocyte-specific Smad4 gene conditional knockout results in hearing loss and inner ear malformation in mice. Dep Dyn 238(8):1897–1908
    • (2009) Dep Dyn , vol.238 , Issue.8 , pp. 1897-1908
    • Yang, S.M.1    Hou, Z.H.2    Yang, G.3    Zhang, J.S.4    Hu, Y.Y.5    Sun, J.H.6    Guo, W.W.7    He, D.8
  • 10
    • 84855858089 scopus 로고    scopus 로고
    • A restricted spectrum of mutations in the SMAD4 tumor-suppressor gene underlies Myhre syndrome
    • COI: 1:CAS:528:DC%2BC38XovFGhug%3D%3D, PID: 22243968
    • Caputo V, Cianetti L, Niceta M, Carta C, Ciolfi A, Bocchinfuso G, Carrani E, Dentici ML et al (2012) A restricted spectrum of mutations in the SMAD4 tumor-suppressor gene underlies Myhre syndrome. Am J Hum Genet 90(1):161–169
    • (2012) Am J Hum Genet , vol.90 , Issue.1 , pp. 161-169
    • Caputo, V.1    Cianetti, L.2    Niceta, M.3    Carta, C.4    Ciolfi, A.5    Bocchinfuso, G.6    Carrani, E.7    Dentici, M.L.8
  • 12
    • 0026029317 scopus 로고
    • Involvement of bone morphogenetic protein-4 (BMP-4) and Vgr-1 in morphogenesis and neurogenesis in the mouse
    • COI: 1:STN:280:DyaK3Mznt1Wmsg%3D%3D, PID: 1893873
    • Jones CM, Lyons KM, Hogan BL (1991) Involvement of bone morphogenetic protein-4 (BMP-4) and Vgr-1 in morphogenesis and neurogenesis in the mouse. Development 111(2):531–542
    • (1991) Development , vol.111 , Issue.2 , pp. 531-542
    • Jones, C.M.1    Lyons, K.M.2    Hogan, B.L.3
  • 13
    • 0034177522 scopus 로고    scopus 로고
    • Vascular endothelial growth factor synergistically enhances bone morphogenetic protein-4-dependent lymphohematopoietic cell generation from embryonic stem cells in vitro
    • COI: 1:CAS:528:DC%2BD3cXitFeitrk%3D, PID: 10733496
    • Nakayama N, Lee J, Chiu L (2000) Vascular endothelial growth factor synergistically enhances bone morphogenetic protein-4-dependent lymphohematopoietic cell generation from embryonic stem cells in vitro. Blood 95(7):2275–2283
    • (2000) Blood , vol.95 , Issue.7 , pp. 2275-2283
    • Nakayama, N.1    Lee, J.2    Chiu, L.3
  • 14
    • 0033942449 scopus 로고    scopus 로고
    • Regulation of BMP/Dpp signaling during embryonic development
    • COI: 1:CAS:528:DC%2BD3cXlsl2gs74%3D, PID: 10950309
    • Nakayama T, Cui Y, Christian JL (2000) Regulation of BMP/Dpp signaling during embryonic development. Cell Mol Life Sci 57(6):943–956
    • (2000) Cell Mol Life Sci , vol.57 , Issue.6 , pp. 943-956
    • Nakayama, T.1    Cui, Y.2    Christian, J.L.3
  • 15
    • 25844486925 scopus 로고    scopus 로고
    • Noggin antagonism of BMP4 signaling controls development of the axial skeleton in the mouse
    • COI: 1:CAS:528:DC%2BD2MXhtVGrsb%2FK, PID: 16122729
    • Wijgerde M, Karp S, McMahon J, McMahon AP (2005) Noggin antagonism of BMP4 signaling controls development of the axial skeleton in the mouse. Dev Biol 286(1):149–157
    • (2005) Dev Biol , vol.286 , Issue.1 , pp. 149-157
    • Wijgerde, M.1    Karp, S.2    McMahon, J.3    McMahon, A.P.4
  • 16
    • 36249013594 scopus 로고    scopus 로고
    • BMP4 induction of sensory neurons from human embryonic stem cells and reinnervation of sensory epithelium
    • PID: 18005071
    • Shi F, Corrales CE, Liberman MC, Edge AS (2007) BMP4 induction of sensory neurons from human embryonic stem cells and reinnervation of sensory epithelium. Eur J Neurosci 26(11):3016–3023
    • (2007) Eur J Neurosci , vol.26 , Issue.11 , pp. 3016-3023
    • Shi, F.1    Corrales, C.E.2    Liberman, M.C.3    Edge, A.S.4
  • 17
    • 0036193069 scopus 로고    scopus 로고
    • Generation of Smad4/Dpc4 conditional knockout mice
    • COI: 1:CAS:528:DC%2BD38XitlWrtLc%3D, PID: 11857783
    • Yang X, Li C, Herrera PL, Deng CX (2002) Generation of Smad4/Dpc4 conditional knockout mice. Genesis 32(2):80–81
    • (2002) Genesis , vol.32 , Issue.2 , pp. 80-81
    • Yang, X.1    Li, C.2    Herrera, P.L.3    Deng, C.X.4
  • 18
    • 0031805156 scopus 로고    scopus 로고
    • CD1 hearing-impaired mice. I: distortion product otoacoustic emission levels, cochlear function and morphology
    • PID: 9667429
    • Le Calvez S, Avan P, Gilain L, Romand R (1998) CD1 hearing-impaired mice. I: distortion product otoacoustic emission levels, cochlear function and morphology. Hear Res 120(1–2):37–50
    • (1998) Hear Res , vol.120 , Issue.1-2 , pp. 37-50
    • Le Calvez, S.1    Avan, P.2    Gilain, L.3    Romand, R.4
  • 19
    • 0035888306 scopus 로고    scopus 로고
    • Localization of the presynaptic cytomatrix protein Piccolo at ribbon and conventional synapses in the rat retina: comparison with Bassoon
    • COI: 1:CAS:528:DC%2BD3MXnvVGjtb0%3D
    • Dick O, Hack I, Altrock WD, Garner CC, Gundelfinger ED, Brandstatter JH (2001) Localization of the presynaptic cytomatrix protein Piccolo at ribbon and conventional synapses in the rat retina: comparison with Bassoon. J Com Neurol 439(2):224–234
    • (2001) J Com Neurol , vol.439 , Issue.2 , pp. 224-234
    • Dick, O.1    Hack, I.2    Altrock, W.D.3    Garner, C.C.4    Gundelfinger, E.D.5    Brandstatter, J.H.6
  • 20
    • 0037421994 scopus 로고    scopus 로고
    • The presynaptic active zone protein bassoon is essential for photoreceptor ribbon synapse formation in the retina
    • COI: 1:CAS:528:DC%2BD3sXitlSrsLo%3D, PID: 12628168
    • Dick O, tom Dieck S, Altrock WD, Ammermuller J, Weiler R, Garner CC, Gundelfinger ED, Brandstatter JH (2003) The presynaptic active zone protein bassoon is essential for photoreceptor ribbon synapse formation in the retina. Neuron 37(5):775–786
    • (2003) Neuron , vol.37 , Issue.5 , pp. 775-786
    • Dick, O.1    tom Dieck, S.2    Altrock, W.D.3    Ammermuller, J.4    Weiler, R.5    Garner, C.C.6    Gundelfinger, E.D.7    Brandstatter, J.H.8
  • 21
    • 0028904022 scopus 로고
    • Expression of AMPA, kainate, and NMDA receptor subunits in cochlear and vestibular ganglia
    • COI: 1:CAS:528:DyaK2MXksFWnuro%3D, PID: 7891171
    • Niedzielski AS, Wenthold RJ (1995) Expression of AMPA, kainate, and NMDA receptor subunits in cochlear and vestibular ganglia. J Neurosci 15(3 Pt 2):2338–2353
    • (1995) J Neurosci , vol.15 , Issue.3 , pp. 2338-2353
    • Niedzielski, A.S.1    Wenthold, R.J.2
  • 22
    • 0029900136 scopus 로고    scopus 로고
    • Organization of AMPA receptor subunits at a glutamate synapse: a quantitative immunogold analysis of hair cell synapses in the rat organ of Corti
    • COI: 1:CAS:528:DyaK28Xktlams7c%3D, PID: 8699256
    • Matsubara A, Laake JH, Davanger S, Usami S, Ottersen OP (1996) Organization of AMPA receptor subunits at a glutamate synapse: a quantitative immunogold analysis of hair cell synapses in the rat organ of Corti. J Neurosci 16(14):4457–4467
    • (1996) J Neurosci , vol.16 , Issue.14 , pp. 4457-4467
    • Matsubara, A.1    Laake, J.H.2    Davanger, S.3    Usami, S.4    Ottersen, O.P.5
  • 24
    • 0142242182 scopus 로고    scopus 로고
    • Cerebellar deficits and hyperactivity in mice lacking Smad4
    • COI: 1:CAS:528:DC%2BD3sXot1amurY%3D, PID: 12896967
    • Zhou YX, Zhao M, Li D, Shimazu K, Sakata K, Deng CX, Lu B (2003) Cerebellar deficits and hyperactivity in mice lacking Smad4. J Biol Chem 278(43):42313–42320
    • (2003) J Biol Chem , vol.278 , Issue.43 , pp. 42313-42320
    • Zhou, Y.X.1    Zhao, M.2    Li, D.3    Shimazu, K.4    Sakata, K.5    Deng, C.X.6    Lu, B.7
  • 25
    • 18044376597 scopus 로고    scopus 로고
    • Recovery of human compound action potential using a paired-click stimulation paradigm
    • PID: 15855044
    • Ohashi T, Ochi K, Nishino H, Kenmochi M, Yoshida K (2005) Recovery of human compound action potential using a paired-click stimulation paradigm. Hear Res 203(1–2):192–200
    • (2005) Hear Res , vol.203 , Issue.1-2 , pp. 192-200
    • Ohashi, T.1    Ochi, K.2    Nishino, H.3    Kenmochi, M.4    Yoshida, K.5
  • 26
    • 67650730447 scopus 로고    scopus 로고
    • Otoferlin interacts with myosin VI: implications for maintenance of the basolateral synaptic structure of the inner hair cell
    • COI: 1:CAS:528:DC%2BD1MXosVagu7s%3D, PID: 19417007
    • Heidrych P, Zimmermann U, Kuhn S, Franz C, Engel J, Duncker SV, Hirt B, Pusch CM et al (2009) Otoferlin interacts with myosin VI: implications for maintenance of the basolateral synaptic structure of the inner hair cell. Hum Mol Genet 18(15):2779–2790
    • (2009) Hum Mol Genet , vol.18 , Issue.15 , pp. 2779-2790
    • Heidrych, P.1    Zimmermann, U.2    Kuhn, S.3    Franz, C.4    Engel, J.5    Duncker, S.V.6    Hirt, B.7    Pusch, C.M.8
  • 27
    • 17244366230 scopus 로고    scopus 로고
    • Hair cell synaptic ribbons are essential for synchronous auditory signalling
    • COI: 1:CAS:528:DC%2BD2MXjtFOmsrs%3D, PID: 15829963
    • Khimich D, Nouvian R, Pujol R, tom Dieck S, Egner A, Gundelfinger ED, Moser T (2005) Hair cell synaptic ribbons are essential for synchronous auditory signalling. Nature 434(7035):889–894
    • (2005) Nature , vol.434 , Issue.7035 , pp. 889-894
    • Khimich, D.1    Nouvian, R.2    Pujol, R.3    tom Dieck, S.4    Egner, A.5    Gundelfinger, E.D.6    Moser, T.7
  • 28
  • 29
    • 78049314066 scopus 로고    scopus 로고
    • Nonneuronal cells regulate synapse formation in the vestibular sensory epithelium via erbB-dependent BDNF expression
    • COI: 1:CAS:528:DC%2BC3cXht1OhurjI, PID: 20837532
    • Gomez-Casati ME, Murtie JC, Rio C, Stankovic K, Liberman MC, Corfas G (2010) Nonneuronal cells regulate synapse formation in the vestibular sensory epithelium via erbB-dependent BDNF expression. Proc Natl Acad Sci U S A 107(39):17005–17010
    • (2010) Proc Natl Acad Sci U S A , vol.107 , Issue.39 , pp. 17005-17010
    • Gomez-Casati, M.E.1    Murtie, J.C.2    Rio, C.3    Stankovic, K.4    Liberman, M.C.5    Corfas, G.6
  • 30
    • 38149120796 scopus 로고    scopus 로고
    • Sensorineural deafness and seizures in mice lacking vesicular glutamate transporter 3
    • COI: 1:CAS:528:DC%2BD1cXhvVSrt7s%3D, PID: 18215623
    • Seal RP, Akil O, Yi E, Weber CM, Grant L, Yoo J, Clause A, Kandler K et al (2008) Sensorineural deafness and seizures in mice lacking vesicular glutamate transporter 3. Neuron 57(2):263–275
    • (2008) Neuron , vol.57 , Issue.2 , pp. 263-275
    • Seal, R.P.1    Akil, O.2    Yi, E.3    Weber, C.M.4    Grant, L.5    Yoo, J.6    Clause, A.7    Kandler, K.8
  • 31
    • 51149113537 scopus 로고    scopus 로고
    • Multiple RIBEYE-RIBEYE interactions create a dynamic scaffold for the formation of synaptic ribbons
    • COI: 1:CAS:528:DC%2BD1cXpvFKku78%3D, PID: 18685021
    • Magupalli VG, Schwarz K, Alpadi K, Natarajan S, Seigel GM, Schmitz F (2008) Multiple RIBEYE-RIBEYE interactions create a dynamic scaffold for the formation of synaptic ribbons. J Neurosci 28(32):7954–7967
    • (2008) J Neurosci , vol.28 , Issue.32 , pp. 7954-7967
    • Magupalli, V.G.1    Schwarz, K.2    Alpadi, K.3    Natarajan, S.4    Seigel, G.M.5    Schmitz, F.6
  • 32
    • 79955158904 scopus 로고    scopus 로고
    • Ribeye is required for presynaptic Ca(V)1.3a channel localization and afferent innervation of sensory hair cells
    • COI: 1:CAS:528:DC%2BC3MXms1Wgu78%3D, PID: 21350006
    • Sheets L, Trapani JG, Mo W, Obholzer N, Nicolson T (2011) Ribeye is required for presynaptic Ca(V)1.3a channel localization and afferent innervation of sensory hair cells. Development 138(7):1309–1319
    • (2011) Development , vol.138 , Issue.7 , pp. 1309-1319
    • Sheets, L.1    Trapani, J.G.2    Mo, W.3    Obholzer, N.4    Nicolson, T.5
  • 33
    • 79957634907 scopus 로고    scopus 로고
    • Functional role of neurotrophin-3 in synapse regeneration by spiral ganglion neurons on inner hair cells after excitotoxic trauma in vitro
    • COI: 1:CAS:528:DC%2BC3MXnt1Cns7Y%3D, PID: 21613508
    • Wang Q, Green SH (2011) Functional role of neurotrophin-3 in synapse regeneration by spiral ganglion neurons on inner hair cells after excitotoxic trauma in vitro. J Neurosci 31(21):7938–7949
    • (2011) J Neurosci , vol.31 , Issue.21 , pp. 7938-7949
    • Wang, Q.1    Green, S.H.2
  • 34
    • 66149125273 scopus 로고    scopus 로고
    • The molecular architecture of ribbon presynaptic terminals
    • COI: 1:CAS:528:DC%2BD1MXivFylt7s%3D, PID: 19253034
    • Zanazzi G, Matthews G (2009) The molecular architecture of ribbon presynaptic terminals. Mol Neurobiol 39(2):130–148
    • (2009) Mol Neurobiol , vol.39 , Issue.2 , pp. 130-148
    • Zanazzi, G.1    Matthews, G.2
  • 35
    • 0033959338 scopus 로고    scopus 로고
    • Addition of the BMP4 antagonist, noggin, disrupts avian inner ear development
    • COI: 1:CAS:528:DC%2BD3cXpvFGrug%3D%3D, PID: 10654599
    • Gerlach LM, Hutson MR, Germiller JA, Nguyen-Luu D, Victor JC, Barald KF (2000) Addition of the BMP4 antagonist, noggin, disrupts avian inner ear development. Development 127(1):45–54
    • (2000) Development , vol.127 , Issue.1 , pp. 45-54
    • Gerlach, L.M.1    Hutson, M.R.2    Germiller, J.A.3    Nguyen-Luu, D.4    Victor, J.C.5    Barald, K.F.6
  • 36
    • 0037378477 scopus 로고    scopus 로고
    • Retinoic acid repression of bone morphogenetic protein 4 in inner ear development
    • COI: 1:CAS:528:DC%2BD3sXisFensLY%3D, PID: 12640113
    • Thompson DL, Gerlach-Bank LM, Barald KF, Koenig RJ (2003) Retinoic acid repression of bone morphogenetic protein 4 in inner ear development. Mol Cell Biol 23(7):2277–2286
    • (2003) Mol Cell Biol , vol.23 , Issue.7 , pp. 2277-2286
    • Thompson, D.L.1    Gerlach-Bank, L.M.2    Barald, K.F.3    Koenig, R.J.4
  • 38
    • 23944442163 scopus 로고    scopus 로고
    • Synaptic transmission at the cochlear nucleus endbulb synapse during age-related hearing loss in mice
    • PID: 15901757
    • Wang Y, Manis PB (2005) Synaptic transmission at the cochlear nucleus endbulb synapse during age-related hearing loss in mice. J Neurophysiol 94(3):1814–1824
    • (2005) J Neurophysiol , vol.94 , Issue.3 , pp. 1814-1824
    • Wang, Y.1    Manis, P.B.2
  • 39
    • 12844256493 scopus 로고    scopus 로고
    • Two ribeye genes in teleosts: the role of Ribeye in ribbon formation and bipolar cell development
    • COI: 1:CAS:528:DC%2BD2MXhtlalt7o%3D, PID: 15673675
    • Wan L, Almers W, Chen W (2005) Two ribeye genes in teleosts: the role of Ribeye in ribbon formation and bipolar cell development. J Neurosci 25(4):941–949
    • (2005) J Neurosci , vol.25 , Issue.4 , pp. 941-949
    • Wan, L.1    Almers, W.2    Chen, W.3
  • 41
    • 14444271321 scopus 로고    scopus 로고
    • Bassoon, a novel zinc-finger CAG/glutamine-repeat protein selectively localized at the active zone of presynaptic nerve terminals
    • COI: 1:CAS:528:DyaK1cXltFGqs7g%3D, PID: 9679147
    • tom Dieck S, Sanmarti-Vila L, Langnaese K, Richter K, Kindler S, Soyke A, Wex H, Smalla KH et al (1998) Bassoon, a novel zinc-finger CAG/glutamine-repeat protein selectively localized at the active zone of presynaptic nerve terminals. J Cell Biol 142(2):499–509
    • (1998) J Cell Biol , vol.142 , Issue.2 , pp. 499-509
    • tom Dieck, S.1    Sanmarti-Vila, L.2    Langnaese, K.3    Richter, K.4    Kindler, S.5    Soyke, A.6    Wex, H.7    Smalla, K.H.8
  • 42
    • 44849114793 scopus 로고    scopus 로고
    • A multicenter study on the prevalence and spectrum of mutations in the otoferlin gene (OTOF) in subjects with nonsyndromic hearing impairment and auditory neuropathy
    • COI: 1:CAS:528:DC%2BD1cXnvFegsbk%3D, PID: 18381613
    • Rodriguez-Ballesteros M, Reynoso R, Olarte M, Villamar M, Morera C, Santarelli R, Arslan E, Meda C et al (2008) A multicenter study on the prevalence and spectrum of mutations in the otoferlin gene (OTOF) in subjects with nonsyndromic hearing impairment and auditory neuropathy. Hum Mutat 29(6):823–831
    • (2008) Hum Mutat , vol.29 , Issue.6 , pp. 823-831
    • Rodriguez-Ballesteros, M.1    Reynoso, R.2    Olarte, M.3    Villamar, M.4    Morera, C.5    Santarelli, R.6    Arslan, E.7    Meda, C.8


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.