【《纤毛的类型结构与相关疾病文献综述》5900字】

纤毛的类型结构与相关疾病文献综述目录TOC\o"1-3"\h\u26520纤毛的类型结构与相关疾病文献综述 1186191.1纤毛的类型与结构 152621.2纤毛的发生 452691.3纤毛的功能 657411.4纤毛的相关疾病 71.1纤毛的类型与结构纤毛是一种进化上保守的细胞器，它几乎存在于所有的真核生物和脊椎动物中，并且广泛分布于不同组织内部，功能各不相同。纤毛由基于细胞骨架微管和动力蛋白所组成，质膜突出于细胞表面，呈现细小毛发状ADDINEN.CITE<EndNote><Cite><Author>Pazour</Author><Year>2003</Year><RecNum>835</RecNum><DisplayText>(PazourandWitman,2003)</DisplayText><record><rec-number>835</rec-number><foreign-keys><keyapp="EN"db-id="vrwdadt98e5druedp2b5e2dc9wz2des0w22f"timestamp="1614428485">835</key></foreign-keys><ref-typename="JournalArticle">17</ref-type><contributors><authors><author>Pazour,G.J.</author><author>Witman,G.B.</author></authors></contributors><auth-address>UnivMassachusetts,SchMed,ProgramMolMed,Worcester,MA01605USA UnivMassachusetts,SchMed,DeptCellBiol,Worcester,MA01605USA</auth-address><titles><title>Thevertebrateprimaryciliumisasensoryorganelle</title><secondary-title>CurrentOpinioninCellBiology</secondary-title><alt-title>CurrOpinCellBiol</alt-title></titles><periodical><full-title>CurrentOpinioninCellBiology</full-title></periodical><alt-periodical><full-title>CurrOpinCellBiol</full-title></alt-periodical><pages>105-110</pages><volume>15</volume><number>1</number><keywords><keyword>polycystickidney-disease</keyword><keyword>left-rightasymmetry</keyword><keyword>rudimentarycilia</keyword><keyword>situs-inversus</keyword><keyword>alpha-tubulin</keyword><keyword>gene</keyword><keyword>protein</keyword><keyword>cells</keyword><keyword>expression</keyword><keyword>mice</keyword></keywords><dates><year>2003</year><pub-dates><date>Feb</date></pub-dates></dates><isbn>0955-0674</isbn><accession-num>WOS:000180581400015</accession-num><urls><related-urls><url><GotoISI>://WOS:000180581400015</url></related-urls></urls><electronic-resource-num>Doi10.1016/S0955-0674(02)00012-1</electronic-resource-num><language>English</language></record></Cite></EndNote>(PazourandWitman,2003)。近年随着对纤毛运动机制的研究、纤毛相关疾病的探究和一些相关蛋白的发现，人们对纤毛的认知也有了新的提高，它不仅可以参与呼吸、生殖、消化等生理过程，纤毛还可与细胞信号感受和转导、个体发育和细胞增殖分化等过程相关ADDINEN.CITEADDINEN.CITE.DATA(Fliegaufetal.,2007;Gerdesetal.,2009;Pan,2008)。纤毛根据其结构特点（中心是否具有中央微管对）和运动特征，可分为四种：“9+2”型轴丝结构的动纤毛（例如，精子鞭毛和消化道上皮细胞的纤毛等），“9+2”型轴丝的静纤毛（例如，内耳细胞中的静纤毛），“9+0”型轴丝结构的静纤毛（例如，视网膜感光细胞、肾小管细胞和感受细胞等），“9+0”型轴丝结构的动纤毛（例如，胚胎的节点细胞）ADDINEN.CITEADDINEN.CITE.DATA(BabuandRoy,2013;FischandDupuis-Williams,2011;VerheyandYang,2016)。纤毛的结构组成和直径上相对保守，其主要有纤毛膜（Ciliarymembrane）、轴丝（Axoneme）、过渡区（Transitionzone）和基体（Basalbody）四部分构成，直径在250nm左右（图1.10）。纤毛膜是蛋白质和脂质成份不同于质膜的一种细胞膜，均匀覆盖于轴丝表面。纤毛膜参与信号转导、感受刺激转导和内外物质交换屏障作用。根据近年研究发现，纤毛膜上蛋白可能是由高尔基将其运输到囊泡，囊泡在纤毛基部发生融合作用，此时，被运输的蛋白成为纤毛膜上不可或缺的组分ADDINEN.CITE<EndNote><Cite><Author>Rohatgi</Author><Year>2010</Year><RecNum>954</RecNum><DisplayText>(RohatgiandSnell,2010)</DisplayText><record><rec-number>954</rec-number><foreign-keys><keyapp="EN"db-id="vrwdadt98e5druedp2b5e2dc9wz2des0w22f"timestamp="1614580443">954</key><keyapp="ENWeb"db-id="">0</key></foreign-keys><ref-typename="JournalArticle">17</ref-type><contributors><authors><author>Rohatgi,R.</author><author>Snell,W.J.</author></authors></contributors><auth-address>DepartmentofMedicine,StanfordUniversitySchoolofMedicine,279CampusDrive,Stanford,CA94305,USA.rrohatgi@</auth-address><titles><title>Theciliarymembrane</title><secondary-title>CurrOpinCellBiol</secondary-title></titles><periodical><full-title>CurrOpinCellBiol</full-title></periodical><pages>541-6</pages><volume>22</volume><number>4</number><edition>2010/04/20</edition><keywords><keyword>Animals</keyword><keyword>Cilia/*metabolism</keyword><keyword>Humans</keyword><keyword>IntracellularMembranes/*metabolism</keyword><keyword>LipidMetabolism</keyword><keyword>MembraneProteins/metabolism</keyword><keyword>ProteinTransport</keyword><keyword>SignalTransduction</keyword></keywords><dates><year>2010</year><pub-dates><date>Aug</date></pub-dates></dates><isbn>1879-0410(Electronic) 0955-0674(Linking)</isbn><accession-num>20399632</accession-num><urls><related-urls><url>/pubmed/20399632</url></related-urls></urls><custom2>PMC2910237</custom2><electronic-resource-num>10.1016/j.ceb.2010.03.010</electronic-resource-num></record></Cite></EndNote>(RohatgiandSnell,2010)。纤毛膜上的脂蛋白主要有Hedgehog（Hh信号通路）、Wingless（Wnt）信号通路和PDGFs受体以及其他各种疾病相关蛋白受体和通道蛋白ADDINEN.CITEADDINEN.CITE.DATA(Huangfuetal.,2003;Lancasteretal.,2011;Schneideretal.,2005;Wardetal.,2003)。轴丝是由微管所组成的纤毛骨架，典型的轴丝是包含呈环状的9组二联体微管和一对中央微管（静纤毛及特色胚胎节点细胞没有中央微管）。轴丝正端指向纤毛的顶端，另一端则连接基体部指向细胞皮层。纤毛的二联体微管是由完整的13根原丝A管和不完整的10根原丝B管所组成，原丝是α-微管蛋白和β-微管蛋白聚合而成的异源二聚体。在“9+2”型纤毛中，内/外动力蛋白臂（inner/outerdyneinarm）分别从二联体微管A管向内外两侧伸出，沿相邻B管滑动，从而使为纤毛运动产生力量。二联体微管是通过放射辐（radialspoke）与中央微管周围的中央鞘相连接ADDINEN.CITEADDINEN.CITE.DATA(GoetzandAnderson,2010;Reiteretal.,2012;VerheyandYang,2016)。图1.10纤毛结构示意图纤毛/鞭毛是一类特殊细胞器。运动纤毛的结构基于9组双联体微管和一对中央微管。双联体微管附着其它结构，如内、外动力臂，径向辐条结构和链接蛋白。节点纤毛位于胚胎节点中，缺少中央对的复合体，存在动力臂，可以完成旋转运动。原生纤毛缺乏中央微管和其它附属结构ADDINEN.CITE<EndNote><Cite><Author>Fisch</Author><Year>2011</Year><RecNum>892</RecNum><DisplayText>(FischandDupuis-Williams,2011)</DisplayText><record><rec-number>892</rec-number><foreign-keys><keyapp="EN"db-id="vrwdadt98e5druedp2b5e2dc9wz2des0w22f"timestamp="1614502599">892</key></foreign-keys><ref-typename="JournalArticle">17</ref-type><contributors><authors><author>Fisch,C.</author><author>Dupuis-Williams,P.</author></authors></contributors><auth-address>INSERMUEVEU829,ATIGECentriole&AssociatedPathol,F-91000Evry,France ESPCI,F-75005Paris,France</auth-address><titles><title>Ultrastructureofciliaandflagella-backtothefuture!</title><secondary-title>BiologyoftheCell</secondary-title><alt-title>BiolCell</alt-title></titles><periodical><full-title>BiologyoftheCell</full-title><abbr-1>BiolCell</abbr-1></periodical><alt-periodical><full-title>BiologyoftheCell</full-title><abbr-1>BiolCell</abbr-1></alt-periodical><pages>249-270</pages><volume>103</volume><number>6</number><keywords><keyword>centralcap</keyword><keyword>cilium</keyword><keyword>flagellum</keyword><keyword>necklace</keyword><keyword>terminalplate</keyword><keyword>transitionzone</keyword><keyword>microtubule-cappingstructures</keyword><keyword>armsin-situ</keyword><keyword>photoreceptorconnectingcilium</keyword><keyword>scanning-electron-microscopy</keyword><keyword>thermophilabasalbodies</keyword><keyword>intraflagellartransport</keyword><keyword>chlamydomonas-reinhardtii</keyword><keyword>cryoelectrontomography</keyword><keyword>fine-structure</keyword><keyword>olfactorycilia</keyword></keywords><dates><year>2011</year><pub-dates><date>Jun</date></pub-dates></dates><isbn>0248-4900</isbn><accession-num>WOS:000293315800001</accession-num><urls><related-urls><url><GotoISI>://WOS:000293315800001</url></related-urls></urls><electronic-resource-num>10.1042/Bc20100139</electronic-resource-num><language>English</language></record></Cite></EndNote>(FischandDupuis-Williams,2011)。在轴丝和基体远端之间有一个区域，称为过渡区（Transitionzone，TZ）。这个区域在电子显微镜下，可以观察到有呈Y型的结构（也称为Y-links），该蛋白一端连接纤毛膜，另一端连接二连体微管。Y型蛋白的具体组成成份还尚不完全清楚。Y-links还同纤毛膜共同组成了一个称为纤毛项圈（ciliarynecklace）的结构ADDINEN.CITE<EndNote><Cite><Author>Gilula</Author><Year>1972</Year><RecNum>960</RecNum><DisplayText>(GilulaandSatir,1972)</DisplayText><record><rec-number>960</rec-number><foreign-keys><keyapp="EN"db-id="vrwdadt98e5druedp2b5e2dc9wz2des0w22f"timestamp="1614594810">960</key></foreign-keys><ref-typename="JournalArticle">17</ref-type><contributors><authors><author>Gilula,N.B.</author><author>Satir,P.</author></authors></contributors><titles><title>Theciliarynecklace.Aciliarymembranespecialization</title><secondary-title>JCellBiol</secondary-title></titles><periodical><full-title>JCellBiol</full-title></periodical><pages>494-509</pages><volume>53</volume><number>2</number><edition>1972/05/01</edition><keywords><keyword>Animals</keyword><keyword>Bivalvia</keyword><keyword>CellMembrane</keyword><keyword>ChickEmbryo</keyword><keyword>*Cilia</keyword><keyword>Eukaryota</keyword><keyword>Fibroblasts/cytology</keyword><keyword>FreezeEtching</keyword><keyword>Gills/cytology</keyword><keyword>HistologicalTechniques</keyword><keyword>Male</keyword><keyword>Microscopy,Electron</keyword><keyword>Microtomy</keyword><keyword>Microtubules</keyword><keyword>Models,Structural</keyword><keyword>Rats</keyword><keyword>SeaUrchins</keyword><keyword>Spermatozoa/cytology</keyword><keyword>Trachea/cytology</keyword></keywords><dates><year>1972</year><pub-dates><date>May</date></pub-dates></dates><isbn>0021-9525(Print) 0021-9525(Linking)</isbn><accession-num>4554367</accession-num><urls><related-urls><url>/pubmed/4554367</url></related-urls></urls><custom2>PMC2108734</custom2><electronic-resource-num>10.1083/jcb.53.2.494</electronic-resource-num></record></Cite></EndNote>(GilulaandSatir,1972)。临近TZ区近端存在一种成份未知的类似纤维结构，称为过渡纤维（transitionfibers，TFs），TFs一端锚定在母中心粒，另一端在质膜和纤毛膜交界处ADDINEN.CITEADDINEN.CITE.DATA(Benmerah,2013;VerheyandYang,2016)。纤毛作为一个独立的细胞器，它内部的蛋白成分与其膜外基质大大不同，这种独立的专属环境非常需要一种门控和运输机制存在。更重要的是门控系统可以根据细胞外的信号调节纤毛蛋白的位置来响应信号通路ADDINEN.CITE<EndNote><Cite><Author>Takao</Author><Year>2016</Year><RecNum>73</RecNum><DisplayText>(TakaoandVerhey,2016)</DisplayText><record><rec-number>73</rec-number><foreign-keys><keyapp="EN"db-id="vrwdadt98e5druedp2b5e2dc9wz2des0w22f"timestamp="1552112791">73</key><keyapp="ENWeb"db-id="">0</key></foreign-keys><ref-typename="JournalArticle">17</ref-type><contributors><authors><author>Takao,D.</author><author>Verhey,K.J.</author></authors></contributors><titles><title>Gatedentryintotheciliarycompartment</title><secondary-title>CellMolLifeSci</secondary-title></titles><periodical><full-title>CellMolLifeSci</full-title></periodical><pages>119-27</pages><volume>73</volume><number>1</number><keywords><keyword>Animals</keyword><keyword>Cilia/*metabolism/ultrastructure</keyword><keyword>Cytosol/metabolism</keyword><keyword>Humans</keyword><keyword>MembraneProteins/metabolism</keyword><keyword>NuclearPoreComplexProteins/*metabolism/ultrastructure</keyword><keyword>ProteinTransport</keyword></keywords><dates><year>2016</year><pub-dates><date>Jan</date></pub-dates></dates><isbn>1420-9071(Electronic) 1420-682X(Linking)</isbn><accession-num>26472341</accession-num><urls><related-urls><url>/pubmed/26472341</url></related-urls></urls><custom2>PMC4959937</custom2><electronic-resource-num>10.1007/s00018-015-2058-0</electronic-resource-num></record></Cite></EndNote>(TakaoandVerhey,2016)。例如，Hh信号通路中的跨膜蛋白Patched,Smoothened和GPR161进入和退出门控系统是由hedgehog配体控制的。细胞外可溶性分子进入纤毛内部是有一定限制的。近期根据建立的不同研究方法发现，有两项研究发现蛋白质大小在40~70kDa进入纤毛内是被限制的，而另一个研究发现更大一些的蛋白也可以非常缓慢的进入ADDINEN.CITEADDINEN.CITE.DATA(Breslowetal.,2013;Keeetal.,2012)。另一项研究根据建立的动力学数学模型发现纤毛屏障作用类比于分子筛，可以允许小于50kDa的蛋白大小以依赖势能的方式自由进出ADDINEN.CITEADDINEN.CITE.DATA(Linetal.,2013;TakaoandVerhey,2016)。纤毛过渡区的特有蛋白对于其功能发挥具有重要的作用，根据蛋白结构和成份这些蛋白主要分为两个类：Nephronophthisis(NPHP)蛋白模块和theMeckelsyndrome（MKS）蛋白模块。如图所示，MKS蛋白模块是主要定位在膜上，与隔膜蛋白一起形成膜蛋白进入的通透性屏障。NPHP类蛋白定位于Y-links，可以同时协同CPC类蛋白和MKS类蛋白ADDINEN.CITE<EndNote><Cite><Author>Verhey</Author><Year>2016</Year><RecNum>82</RecNum><DisplayText>(VerheyandYang,2016)</DisplayText><record><rec-number>82</rec-number><foreign-keys><keyapp="EN"db-id="vrwdadt98e5druedp2b5e2dc9wz2des0w22f"timestamp="1552298889">82</key><keyapp="ENWeb"db-id="">0</key></foreign-keys><ref-typename="JournalArticle">17</ref-type><contributors><authors><author>Verhey,K.J.</author><author>Yang,W.</author></authors></contributors><auth-address>DepartmentofCellandDevelopmentalBiology,UniversityofMichiganMedicalSchool,AnnArbor,MI48109,USA.Electronicaddress:kjverhey@. DepartmentofBiology,TempleUniversity,Philadelphia,PA19122,USA.</auth-address><titles><title>Permeabilitybarriersforgeneratingauniqueciliaryproteinandlipidcomposition</title><secondary-title>CurrOpinCellBiol</secondary-title></titles><periodical><full-title>CurrOpinCellBiol</full-title></periodical><pages>109-16</pages><volume>41</volume><keywords><keyword>Animals</keyword><keyword>Cilia/*metabolism</keyword><keyword>Humans</keyword><keyword>Lipids/*chemistry</keyword><keyword>Models,Biological</keyword><keyword>Permeability</keyword><keyword>Proteins/*metabolism</keyword></keywords><dates><year>2016</year><pub-dates><date>Aug</date></pub-dates></dates><isbn>1879-0410(Electronic) 0955-0674(Linking)</isbn><accession-num>27232950</accession-num><urls><related-urls><url>/pubmed/27232950</url></related-urls></urls><custom2>PMC4983468</custom2><electronic-resource-num>10.1016/j.ceb.2016.05.004</electronic-resource-num></record></Cite></EndNote>(VerheyandYang,2016)。生化和遗传学分析将这些蛋白分为三个功能模块:MKS、NPHP1-4-8和NPHP5-6。MKS复合物在纤毛虫中是保守的，而NPHP1-4-8和NPHP5-6复合物可能以一种物种特异性或细胞特异性的方式与MKS复合物相互作用ADDINEN.CITEADDINEN.CITE.DATA(Barkeretal.,2014;Basirietal.,2014;Sangetal.,2011)。图1.11纤毛中纤毛门结构示意图纤毛门是纤毛特化的结构，具有Y-linker结构，具体的蛋白成分包括MKS和NPHPD等，所有纤毛的轴丝从基体结构延伸出来。其中Y-link结构组织或者构成纤毛表面的项链突起。线虫中基体在纤毛形成后几乎完全退化，只保留过渡纤维。1.2纤毛的发生纤毛的发生是一个连续、动态的过程，这个过程由膜动力学、微管动力学和肌动蛋白动力学相互协调合作完成，最终形成一个细胞器ADDINEN.CITE<EndNote><Cite><Author>Snyder</Author><Year>2020</Year><RecNum>1272</RecNum><DisplayText>(Snyderetal.,2020)</DisplayText><record><rec-number>1272</rec-number><foreign-keys><keyapp="EN"db-id="vrwdadt98e5druedp2b5e2dc9wz2des0w22f"timestamp="1616927929">1272</key></foreign-keys><ref-typename="JournalArticle">17</ref-type><contributors><authors><author>Snyder,R.</author><author>Verla,T.</author><author>Ropper,A.E.</author></authors></contributors><auth-address>BaylorCollMed,DeptNeurosurg,Houston,TX77030USA</auth-address><titles><title>PracticalApplicationofRecentAdvancesinDiagnostic,Prognostic,andTherapeuticModalitiesforSpinalCordInjury</title><secondary-title>WorldNeurosurgery</secondary-title><alt-title>WorldNeurosurg</alt-title></titles><periodical><full-title>WorldNeurosurgery</full-title><abbr-1>WorldNeurosurg</abbr-1></periodical><alt-periodical><full-title>WorldNeurosurgery</full-title><abbr-1>WorldNeurosurg</abbr-1></alt-periodical><pages>330-336</pages><volume>136</volume><keywords><keyword>engineeredneuroplasticity</keyword><keyword>neuralbypass</keyword><keyword>neurorehabilitation</keyword><keyword>prognosticbiomarkers</keyword><keyword>spinalcordinjury</keyword><keyword>cerebrospinal-fluidbiomarkers</keyword><keyword>intraspinalpressure</keyword><keyword>perfusion-pressure</keyword><keyword>h-reflex</keyword><keyword>exercise</keyword><keyword>severity</keyword><keyword>recovery</keyword><keyword>nerve</keyword><keyword>stimulation</keyword><keyword>profile</keyword></keywords><dates><year>2020</year><pub-dates><date>Apr</date></pub-dates></dates><isbn>1878-8750</isbn><accession-num>WOS:000520838600138</accession-num><urls><related-urls><url><GotoISI>://WOS:000520838600138</url></related-urls></urls><electronic-resource-num>10.1016/j.wneu.2020.01.011</electronic-resource-num><language>English</language></record></Cite></EndNote>(Snyderetal.,2020)。纤毛的发生过程根据细胞种类的不同，分为两种途径：胞内途径和胞外途径ADDINEN.CITEADDINEN.CITE.DATA(Ghossoubetal.,2011;Sorokin,1962)。上皮细胞的纤毛发生属于胞外途径，根据电镜观察结果显示，这个途径中纤毛基体直接同顶端膜相融合，纤毛中的微管则直接组装延伸至细胞外。而成纤维细胞或平滑肌细胞纤毛使用的是胞内途径，这个途径是从细胞内部深处开始，其标志过程是母中心粒的远端原发性囊泡的出现，但是母中心粒是选择一种具有纤毛发生特异性的囊泡连接还是选择一种普通的囊泡连接的机制还是未知的。这个阶段完成了中心粒向基体的转化ADDINEN.CITEADDINEN.CITE.DATA(Qin,2012;Sorokin,1968)。在初级囊泡出现后，会招募更多的囊泡聚集在基体周围，图1.12基体和过渡区在初级纤毛形成过程中的作用在哺乳动物细胞中，纤毛发生的起始事件是在中心粒囊泡（centriolarvesicle，CV）和母中心粒连接开始。（1）中心粒囊泡开始内陷，TZ区域开始出现，膜表面通过二级囊泡融合生长。（2）-（4）带有中心粒的囊泡可以迁移到细胞质膜上，并且发生融合，随后在这个位置上形成纤毛门结构。（5）轴突和纤毛功能的形成是一个IFT(intraflagellartransport)/BBS(Bardet–Biedlsyndrome)蛋白依赖的过程。（6）IFT/BBS蛋白存在于未牟定的基体上，可能是发挥特定的物质运输作用，也可能是只是作为功能性IFT颗粒运输的最终组装。（7）其它类型细胞不同的发生途经，这个过程可能不涉及CV，而是基体直接和细胞膜对接，TZ(transitionzone)区的形成可能在这个过程之前形成，也有可能是在过程之中或者之后。另外，基体的迁移并不是会发生在所有类型的细胞中ADDINEN.CITE<EndNote><Cite><Author>Reiter</Author><Year>2012</Year><RecNum>959</RecNum><DisplayText>(Reiteretal.,2012)</DisplayText><record><rec-number>959</rec-number><foreign-keys><keyapp="EN"db-id="vrwdadt98e5druedp2b5e2dc9wz2des0w22f"timestamp="1614589024">959</key></foreign-keys><ref-typename="JournalArticle">17</ref-type><contributors><authors><author>Reiter,J.F.</author><author>Blacque,O.E.</author><author>Leroux,M.R.</author></authors></contributors><auth-address>DepartmentofBiochemistry&Biophysics,CardiovascularResearchInstitute,UniversityofCalifornia,SanFrancisco,555MissionBayBlvdSouth,California94158,USA.</auth-address><titles><title>Thebaseofthecilium:rolesfortransitionfibresandthetransitionzoneinciliaryformation,maintenanceandcompartmentalization</title><secondary-title>EMBORep</secondary-title></titles><periodical><full-title>EMBORep</full-title></periodical><pages>608-18</pages><volume>13</volume><number>7</number><edition>2012/06/02</edition><keywords><keyword>Animals</keyword><keyword>Axoneme/chemistry/*metabolism/ultrastructure</keyword><keyword>CellCompartmentation</keyword><keyword>Cilia/chemistry/*physiology/ultrastructure</keyword><keyword>CytoplasmicStreaming</keyword><keyword>CytoskeletalProteins/*metabolism</keyword><keyword>Humans</keyword></keywords><dates><year>2012</year><pub-dates><date>Jun29</date></pub-dates></dates><isbn>1469-3178(Electronic) 1469-221X(Linking)</isbn><accession-num>22653444</accession-num><urls><related-urls><url>/pubmed/22653444</url></related-urls></urls><custom2>PMC3388784</custom2><electronic-resource-num>10.1038/embor.2012.73</electronic-resource-num></record></Cite></EndNote>(Reiteretal.,2012)。基体和小泡一起迁移至细胞表面特定的位置后，原生小泡会和质膜发生融合，这个过程依赖于肌动蛋白细胞骨架，同时也需要一些过渡区蛋白的参与，例如MKS1和MKS3ADDINEN.CITEADDINEN.CITE.DATA(Daweetal.,2009;Daweetal.,2007;Lemulloisetal.,1988)。这个是基体细胞质膜对接的关键步骤。在基体与质膜对接后，基体的远端向外伸长形成过渡区（transitionzone，TZ），这是一个结构特征不明显但是非常重要的结构组分。TZ区在进化上相对保守，其特征是该区域横截面显示独特的Y形纤维，连接着二联体微管和纤毛膜。Y形连接在过渡纤维的远端，可以通过纤毛超微结构的切片中观察到ADDINEN.CITE<EndNote><Cite><Author>Andersen</Author><Year>1991</Year><RecNum>1308</RecNum><DisplayText>(Andersenetal.,1991)</DisplayText><record><rec-number>1308</rec-number><foreign-keys><keyapp="EN"db-id="vrwdadt98e5druedp2b5e2dc9wz2des0w22f"timestamp="1617073102">1308</key></foreign-keys><ref-typename="JournalArticle">17</ref-type><contributors><authors><author>Andersen,R.A.</author><author>Barr,D.J.S.</author><author>Lynn,D.H.</author><author>Melkonian,M.</author><author>Moestrup,O.</author><author>Sleigh,M.A.</author></authors></contributors><auth-address>BiosystResInst,OttawaK1a0c6,Ontario,Canada UnivGuelph,DeptZool,GuelphN1g2w1,Ontario,Canada UnivCologne,InstBot,W-5000Cologne41,Germany UnivSouthampton,DeptBiol,SouthamptonSo93tu,Hants,England UnivCopenhagen,InstSporeplanter,Dk-1168Copenhagen,Denmark</auth-address><titles><title>TerminologyandNomenclatureoftheCytoskeletalElementsAssociatedwiththeFlagellarCiliaryApparatusinProtists</title><secondary-title>Protoplasma</secondary-title><alt-title>Protoplasma</alt-title></titles><periodical><full-title>Protoplasma</full-title></periodical><alt-periodical><full-title>Protoplasma</full-title></alt-periodical><pages>1-8</pages><volume>164</volume><number>1-3</number><keywords><keyword>flagellateciliate</keyword><keyword>protists</keyword><keyword>cytoskeleton</keyword><keyword>flagellarapparatus</keyword><keyword>kinetid</keyword><keyword>terminology</keyword><keyword>nomenclature</keyword></keywords><dates><year>1991</year></dates><isbn>0033-183x</isbn><accession-num>WOS:A1991GU47700001</accession-num><urls><related-urls><url><GotoISI>://WOS:A1991GU47700001</url></related-urls></urls><electronic-resource-num>Doi10.1007/Bf01320809</electronic-resource-num><language>English</language></record></Cite></EndNote>(Andersenetal.,1991)。目前，人们对Y形连接结构的组成基本未知，但是特定过渡区定位和功能相关蛋白可能是Y形连接结构的候选组分，包括与Meckel综合症(MKS)、Joubert综合症(JBTS)、肾性眼病(NPHP)、Senior-Loken综合症(SLSN)和Leber-先天性黑内障(LCA)有关的纤毛病蛋白。在TZ区形成后，基体内的三联体微管转化为外联体微管。9组二联体微管是初级纤毛轴突的主要组成部分。在动纤毛中存在帮助运动的附属结构，例如，二联体微管中心部分还存在一对中央微管，以及二联体微管周围的精细有序的内外动力蛋白臂和放射辐条。纤毛轴突的后续生长延长只发生在纤毛的顶端ADDINEN.CITE<EndNote><Cite><Author>Johnson</Author><Year>1992</Year><RecNum>1283</RecNum><DisplayText>(JohnsonandRosenbaum,1992)</DisplayText><record><rec-number>1283</rec-number><foreign-keys><keyapp="EN"db-id="vrwdadt98e5druedp2b5e2dc9wz2des0w22f"timestamp="1617001320">1283</key></foreign-keys><ref-typename="JournalArticle">17</ref-type><contributors><authors><author>Johnson,K.A.</author><author>Rosenbaum,J.L.</author></authors></contributors><auth-address>DepartmentofBiology,YaleUniversity,NewHaven,Connecticut06511.</auth-address><titles><title>Replicationofbasalbodiesandcentrioles</title><secondary-title>CurrOpinCellBiol</secondary-title></titles><periodical><full-title>CurrOpinCellBiol</full-title></periodical><pages>80-5</pages><volume>4</volume><number>1</number><edition>1992/02/01</edition><keywords><keyword>Animals</keyword><keyword>Centrioles/*ultrastructure</keyword><keyword>*DNAReplication</keyword><keyword>Microtubules/ultrastructure</keyword><keyword>Organelles/*ultrastructure</keyword><keyword>SpindleApparatus/ultrastructure</keyword></keywords><dates><year>1992</year><pub-dates><date>Feb</date></pub-dates></dates><isbn>0955-0674(Print) 0955-0674(Linking)</isbn><accession-num>1558756</accession-num><urls><related-urls><url>/pubmed/1558756</url></related-urls></urls><electronic-resource-num>10.1016/0955-0674(92)90062-h</electronic-resource-num></record></Cite></EndNote>(JohnsonandRosenbaum,1992)。在纤毛轴突的生长尖端是纤毛帽结构。电镜结果显示纤毛帽结构首先出现在纤毛轴突和过渡区之间。因此，纤毛帽结构在稳定纤毛结构中起着重要的作用ADDINEN.CITEADDINEN.CITE.DATA(Dentler,1980;Portmanetal.,1987)。纤毛轴丝的延长依赖于纤毛内运输（intraflagellartransport，IFT）。纤毛内部没有蛋白质合成机器核糖体，因此外界物质进入纤毛内和去除废弃的蛋白都需要IFT机制，帮助蛋白质穿越“纤毛门控”系统运输至纤毛的顶端或者运出纤毛。IFT是一种位于二联体微管和纤毛膜之间的双向运输系统，在线虫、四膜虫、锥虫和小鼠肾脏细胞中均可以观察到ADDINEN.CITEADDINEN.CITE.DATA(Jiangetal.,2015;Santi-Roccaetal.,2015)。IFT机制中驱动蛋白（kinesin-2）主要负责将轴突前体物质和微管蛋白等由纤毛基部运输至纤毛顶部进行组装，而动力蛋白(dynein)主要负责将物质逆向运输至纤毛基体ADDINEN.CITE<EndNote><Cite><Author>Hou</Author><Year>2015</Year><RecNum>1292</RecNum><DisplayText>(HouandWitman,2015)</DisplayText><record><rec-number>1292</rec-number><foreign-keys><keyapp="EN"db-id="vrwdadt98e5druedp2b5e2dc9wz2des0w22f"timestamp="1617012527">1292</key></foreign-keys><ref-typename="JournalArticle">17</ref-type><contributors><authors><author>Hou,Y.</author><author>Witman,G.B.</author></authors></contributors><auth-address>DepartmentofCellandDevelopmentalBiology,UniversityofMassachusettsMedicalSchool,Worcester,MA01655,UnitedStates. DepartmentofCellandDevelopmentalBiology,UniversityofMassachusettsMedicalSchool,Worcester,MA01655,UnitedStates.Electronicaddress:George.Witman@.</auth-address><titles><title>Dyneinandintraflagellartransport</title><secondary-title>ExpCellRes</secondary-title></titles><periodical><full-title>ExperimentalCellResearch</full-title><abbr-1>ExpCellRes</abbr-1></periodical><pages>26-34</pages><volume>334</volume><number>1</number><edition>2015/03/01</edition><keywords><keyword>BiologicalTransport</keyword><keyword>Dyneins/*metabolism</keyword><keyword>Flagella/*metabolism</keyword><keyword>Humans</keyword><keyword>Ciliopathy</keyword><keyword>Cytoplasmicdynein2</keyword><keyword>Intraflagellartransport</keyword></keywords><dates><year>2015</year><pub-dates><date>May15</date></pub-dates></dates><isbn>1090-2422(Electronic) 0014-4827(Linking)</isbn><accession-num>25725253</accession-num><urls><related-urls><url>/pubmed/25725253</url></related-urls></urls><custom2>PMC4433774</custom2><electronic-resource-num>10.1016/j.yexcr.2015.02.017</electronic-resource-num></record></Cite></EndNote>(HouandWitman,2015)。IFT颗粒主要包含两类，分别为复合物A（包含六种组分：IFT144,140,139,122,121and43）和复合物B（包含十六种组分：IFT-172,88,81,80,74,70,57,56,54,52,46,38,27,25,22和20）ADDINEN.CITEADDINEN.CITE.DATA(Luckeretal.,2005;Taschneretal.,2011;Taschneretal.,2016)。kinesin-2主要是承担复合物B的运输。dynein主要是承担复合物A的运输。IFT火车是首先在衣藻中观察到的，随后在其它类型的纤毛中也观察到了类似结构，例如视网膜感光细胞ADDINEN.CITE<EndNote><Cite><Author>Rosenbaum</Author><Year>1999</Year><RecNum>1300</RecNum><DisplayText>(Rosenbaumetal.,1999)</DisplayText><record><rec-number>1300</rec-number><foreign-keys><keyapp="EN"db-id="vrwdadt98e5druedp2b5e2dc9wz2des0w22f"timestamp="1617068604">1300</key></foreign-keys><ref-typename="JournalArticle">17</ref-type><contributors><authors><author>Rosenbaum,J.L.</author><author>Cole,D.G.</author><author>Diener,D.R.</author></authors></contributors><auth-address>YaleUniv,DeptMolCellular&DevBiol,NewHaven,CT06520USA</auth-address><titles><title>Intraflagellartransport:Theeyeshaveit</title><secondary-title>JournalofCellBiology</secondary-title><alt-title>JCellBiol</alt-title></titles><periodical><full-title>JournalofCellBiology</full-title></periodical><alt-periodical><full-title>JCellBiol</full-title></alt-periodical><pages>385-388</pages><volume>144</volume><number>3</number><keywords><keyword>kinesin-likeprotein</keyword><keyword>chlamydomonasfla10gene</keyword><keyword>dyneinlight-chain</keyword><keyword>caenorhabditis-elegans</keyword><keyword>flagellarmembrane</keyword><keyword>homologousprotein</keyword><keyword>reinhardtii</keyword><keyword>expression</keyword><keyword>axonemes</keyword><keyword>ciliary</keyword></keywords><dates><year>1999</year><pub-dates><date>Feb8</date></pub-dates></dates><isbn>0021-9525</isbn><accession-num>WOS:000082932500002</accession-num><urls><related-urls><url><GotoISI>://WOS:000082932500002</url></related-urls></urls><electronic-resource-num>DOI10.1083/jcb.144.3.385</electronic-resource-num><language>English</language></record></Cite></EndNote>(Rosenbaumetal.,1999)。IFT火车的大小变化范围很大，最长的约0.7μm，呈40nm周期性；最短约0.25μm，呈16nm周期性。同时在对衣藻的研究中发现，IFT火车的长度同纤毛长度成反比ADDINEN.CITEADDINEN.CITE.DATA(Engeletal.,2009;Vannuccinietal.,2016)。近期，利用光镜-电镜相结合（Correlativelightandelectronmicroscopy,CLEM）的方法观察到，正向和反向的IFT火车长度类似，约0.2μm；正向的IFT火车沿着二联体微管中的B管，并且边界清楚、含有规则的电子致密物质；反向的IFT火车沿着二联体微管中的A管，含有不规则的电子致密物质，结构略松散ADDINEN.CITE<EndNote><Cite><Author>Stepanek</Author><Year>2016</Year><RecNum>1296</RecNum><DisplayText>(StepanekandPigino,2016)</DisplayText><record><rec-number>1296</rec-number><foreign-keys><keyapp="EN"db-id="vrwdadt98e5druedp2b5e2dc9wz2des0w22f"timestamp="1617068284">1296</key></foreign-keys><ref-typename="JournalArticle">17</ref-type><contributors><authors><author>Stepanek,L.</author><author>Pigino,G.</author></authors></contributors><auth-address>MaxPlanckInstituteofMolecularCellBiologyandGenetics(MPI-CBG),Pfotenhauerstrasse108,01307Dresden,German