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耳蜗毛细胞(Hair Cells, HCs)通过顶部纤毛的运动将声信号转换为听神经纤维电信号,通过脑干听觉核团等听觉中枢传导至听觉皮质而产生听觉。目前,应用膜片钳电生理技术,证实了毛细胞膜上可表达多种离子通道及转运蛋白等,而各种病理状态下的HCs均伴有某些特定离子通道的电变化。对这些电变化的研究,将为我们明确许多遗传性耳聋和听觉损伤复杂的发病机理,进一步拓展在细胞以及分子水平上诊断和治疗许多由内耳疾病所导致的耳聋和听力损伤新理念、新思路。
Abstract：Cochlear hair cells(HCs) convert acoustic signals into electrical signals through stereocillia movement to be transmitted to the auditory cortex via the auditory nerve and auditory centers(such as the auditory nucleus in the brain stem) to generate auditory perception. At present, studies using patch clamp technology have shown expression of various ion channels and transporters on HC membrane, and changes of electrical characteristics of certain ion channels associated with HCs under various pathological conditions. Studying these electrical changes provides us with a clear understanding of the complex pathogenesis of hereditary deafness and hearing impairment, and foster new ideas and innovation in the diagnosis and treatment of inner ear diseases at cellular and molecular levels.
1 Lang F,Vallon V,Knipper M,et al.Functional significance of channels and transporters expressed in the inner ear and kidney[J].Am J Physiol Cell Physiol,2007.293(4):C1187-1208.
2 Zdebik AA,Wangemann P,Jentsch TJ.Potassium ion movement in the inner ear:insights from genetic disease and mouse models[J].Physiology (Bethesda),2009.24:307-316.
3 Kharkovets T,Dedek K,Maier H,et al.Mice with altered KC‐NQ4 K+channels implicate sensory outer hair cells in human progressive deafness[J].EMBO J,2006.25(3):642-52.4.Wasano K,Mutai H,Obuchi C,et al.A novel frameshift muta‐tion in KCNQ4 in a family with autosomal recessive non-syn‐dromic hearing loss[J].Biochem Biophys Res Commun,2015.463(4):582-586.
5 Mittal R,Aranke M,Debs LH,et al.Indispensable Role of Ion Channels and Transporters in the Auditory System[J].Journal of Cellular Physiology,2017.232(4):743-758.
6 Molina L,Fasquelle L,Nouvian R,et al.Tmprss3 loss of func‐tion impairs cochlear inner hair cell Kcnma1 channel membrane expression[J].Hum Mol Genet,2013.22(7):1289-1299.
7 Surguchev A,Bai JP,Joshi P,et al.Hair cell BK channels inter‐act with RACK1,and PKC increases its expression on the cell surface by indirect phosphorylation[J].Am J Physiol Cell Physi‐ol,2012.303(2):C143-150.
8 Yu T,Deng C,Wu R,et al.Decreased expression of small-con‐ductance Ca2+-activated K+channels SK1 and SK2 in human chronic atrial fibrillation[J].Life Sci,2012.90(5-6):219-227.
9 Maison SF,Parker LL,Young L,et al.Overexpression of SK2channels enhances efferent suppression of cochlear responses without enhancing noise resistance[J].J Neurophysiol,2007.97(4):2930-2936.
10 Eckrich T,Varakina K,Johnson SL,et al.Development and func‐tion of the voltage-gated sodium current in immature mammali‐an cochlear inner hair cells[J].PLo S One,2012.7(9):e45732.
11 Picher MM,Gehrt A,Meese S,et al.Ca2+-binding protein 2 in‐hibits Ca2+-channel inactivation in mouse inner hair cells[J].Proc Natl Acad Sci U S A,2017.114(9):E1717-E1726.
12 Joiner ML,Lee A.Voltage-gated Cav1 channels in disorders of vision and hearing[J].Curr Mol Pharmacol,2015.8(2):143-148.
13 Weiler S,Krinner S,Wong AB,et al.ATP hydrolysis is critically required for function of Ca V1.3 channels in cochlear inner hair cells via fueling Ca2+clearance[J].J Neurosci,2014.34(20):6843-6848.
14 Vincent PF,Bouleau Y,Charpentier G,et al.Different Ca V1.3channel isoforms control distinct components of the synaptic ves‐icle cycle in auditory inner hair cells[J].J Neurosci,2017.37(11):2960-2975.
15 Monzani D,Genovese E,Pini LA,et al.Nimodipine in otolaryn‐gology:from past evidence to clinical perspectives[J].Acta Oto‐rhinolaryngol Ital,2015.35(3):135-145.
16 Mammano F,Bortolozzi M,Ortolano S,et al.Ca2+Signaling in the Inner Ear[J].Physiology,2007.22(2):131-144.
17 Xie DH,Xiao ZA,Yang S.Acetylcholine-induced calcium oscil‐lation in isolated outer hair cells in guinea pig[J].Journal of Otology,2006.1(2):99-102
18 Clapham DE,Julius D,Montell C,et al.International Union of Pharmacology.XLIX.Nomenclature and structure-function rela‐tionships of transient receptor potential channels[J].Pharmacol Rev,2005.57(4):427-450.
19 Huang CL.The transient receptor potential superfamily of ion channels[J].J Am Soc Nephrol,2004.15(7):1690-1699.
20 Zanini D,G?pfert MC.TRPs in hearing.Handb Exp Pharmacol[J],2014.223:899-916.
21 Kwan KY,Allchorne AJ,Vollrath MA,et al.TRPA1 contributes to cold,mechanical,and chemical nociception but is not essen‐tial for hair-cell transduction[J].Neuron,2006.50(2):277-289.
22 Wu X,Indzhykulian AA,Niksch PD,et al.Hair-cell mechano‐transduction persists in TRP channel knockout mice[J].PLo SOne,2016.11(5):e0155577.
23 Sexton JE,Desmonds T,Quick K,et al.Wood The contribution of TRPC1,TRPC3,TRPC5 and TRPC6 to touch and hearing[J].Neurosci Lett,2016.610:36-42.
24 Gu C,Li X,Tan Q,et al.Mi R-183 family regulates chloride in‐tracellular channel 5 expression in inner ear hair cells[J].Toxi‐col In Vitro,2013.27(1):486-491.
25 Men Y,Li X,Tu H,et al.Tprn is essential for the integrity of ste‐reociliary rootlet in cochlear hair cells in mice[J].Frontiers of Medicine,2018.
26 Zheng J,Shen W,He DZ,et al.Prestin is the motor protein of co‐chlear outer hair cells[J].Nature,2000.405(6783):149-155.
27 Belyantseva IA,Adler HJ,Curi R,et al.Expression and localiza‐tion of prestin and the sugar transporter GLUT-5 during develop‐ment of electromotility in cochlear outer hair cells[J].J Neuro‐sci,2000.20(24):RC116.
28 Liberman MC,Gao J,He DZ,et al.Prestin is required for elec‐tromotility of the outer hair cell and for the cochlear amplifier[J].Nature,2002.419(6904):300-304.
29 Parham,K.Prestin as a biochemical marker for early detection of acquired sensorineural hearing loss[J].Med Hypotheses,2015.85(2):130-133.
30 Oliver D,He DZ,Kl?cker N,et al.Intracellular anions as the voltage sensor of prestin,the outer hair cell motor protein[J].Science,2001.292(5525):2340-2343.
31 Wang J,Li X,Zhang Z,et al.Expression of prestin in OHCs is reduced in Spag6 gene knockout mice[J].Neurosci Lett,2015.
592:42-47.
32 Harasztosi C,Gummer AW.The chloride-channel blocker 9-an‐thracenecarboxylic acid reduces the nonlinear capacitance of prestin-associated charge movement[J].Eur J Neurosci,2016.43(8):1062-1074.
33 Moser T,Beutner D.Kinetics of exocytosis and endocytosis at the cochlear inner hair cell afferent synapse of the mouse[J].Proc Natl Acad Sci U S A,2000.97(2):883-888.
34 Glowatzki E,Fuchs PA.Transmitter release at the hair cell rib‐bon synapse[J].Nat Neurosci,2002.5(2):147-154.
35申卫东,韩东一.内毛细胞突触复合体的结构和功能研究进展[J].中华耳科学杂志,2004.2(2):142-147.Sheng WD,Han DY.Research progress in the structure and func‐tion of the synaptic complex of inner hair cells[J].Chinese Jour‐nal of Otology,2004.2(2):142-147.
36 Grant L,Yi E,Glowatzki E.Two modes of release shape the postsynaptic response at the inner hair cell ribbon synapse[J].JNeurosci,2010.30(12):4210-4220.
37 Graydon CW,Cho S,Li GL,et al.Sharp Ca2?nanodomains be‐neath the ribbon promote highly synchronous multivesicular re‐lease at hair cell synapses[J].J Neurosci,2011.31(46):16637-16650.
38 Li GL,Cho S,von Gersdorff H.Phase-locking precision is en‐hanced by multiquantal release at an auditory hair cell ribbon synapse[J].Neuron,2014.83(6):1404-1417.
39 Magistretti J,Spaiardi P,Johnson SL,et al.Elementary properties of Ca(2+) channels and their influence on multivesicular release and phase-locking at auditory hair cell ribbon synapses[J].Front Cell Neurosci,2015.9:123.
40 Huang CH,Moser T.Ca2+Regulates the Kinetics of Synaptic Vesicle Fusion at the Afferent Inner Hair Cell Synapse[J].Front Cell Neurosci,2018.12:364.
41 Kazmierczak P,Sakaguchi H,Tokita J,et al.Cadherin 23 and protocadherin 15 interact to form tip-link filaments in sensory hair cells[J].Nature,2007.449(7158):87-91.
42 Siemens J,Lillo C,Dumont RA,et al.Cadherin 23 is a compo‐nent of the tip link in hair-cell stereocilia[J].Nature,2004.428(6986):950-955.
43 Alagramam KN,Murcia CL,Kwon HY,et al.The mouse Ames waltzer hearing-loss mutant is caused by mutation of Pcdh15,a novel protocadherin gene[J].Nat Genet,2001.27(1):99-102.
44 Longo-Guess CM,Gagnon LH,Cook SA,et al.A missense mu‐tation in the previously undescribed gene Tmhs underlies deaf‐ness in hurry-scurry (hscy) mice[J].Proc Natl Acad Sci U S A,2005.102(22):7894-7899.
45 Kurima K,Peters LM,Yang Y,et al.Dominant and recessive deafness caused by mutations of a novel gene,TMC1,required for cochlear hair-cell function[J].Nat Genet,2002.30(3):277-284.
46 Kawashima Y,Géléoc GS,Kurima K,et al.Mechanotransduc‐tion in mouse inner ear hair cells requires transmembrane chan‐nel-like genes[J].J Clin Invest,2011.121(12):4796-4809.
47 Bo Zhao,Zizhen Wu,Nicolas Grillet,et al.TMIE is an essential component of the mechanotransduction machinery of cochlear hair cells[J].Neuron,2014.84(5):954-967.
48 Pan B,Akyuz N,Liu XP,et al.TMC1 forms the pore of mecha‐nosensory transduction channels in vertebrate inner ear hair cells[J].Neuron,2018.99(4):736-753.
49 Monesson-Olson BD,Browning-Kamins J,Aziz-Bose R,et al.Optical stimulation of zebrafish hair cells expressing channelrho‐dopsin-2[J].PLo S One,2014.9(5):e96641.
50 Wu T,Ramamoorthy S,Wilson T,et al.Optogenetic control of mouse outer hair cells[J].Biophys J,2016.110(2):493-502.
51 Troconis EL,Ordoobadi AJ,Sommers TF,et al.Intensity-depen‐dent timing and precision of startle response latency in larval ze‐brafish[J].J Physiol,2017.595(1):265-282.
基本信息:
中图分类号:R764.3
引用信息:
[1]田超永,查定军.耳蜗毛细胞的细胞电生理实验研究进展[J].中华耳科学杂志,2020,18(06):1091-1095.
基金信息:
国家自然科学基金面上项目(81870732);; 陕西省科技重点项目(2018PT-01);; 西京助推(XJZT14X07,XJZT15D02,XJZT18MDT07,XJZT18X23,XJZT18D16)~~
2020-12-15
2020-12-15