The distal appendage proteins recruit TTBK2

C2CD3 and the distal appendage protein CEP164 are required for the recruitment of the kinase Tau tubulin kinase 2 (TTBK2) to the centriole (Ye et al, 2014; Cajanek et al, 2014). TTBK2 recruitment promotes the release of CCP110, a negative regulator of ciliogenesis that caps the mother centriole (Goetz et al, 2012; Ye et al, 2014; Tsang et al, 2008; Spektor et al, 2007). CCP110 is initially recruited and/or stabilized at the mother centriole in a KIF24-dependent manner; KIF24, a kinesin-like protein, also restricts ciliogenesis through its microtubule depolymerizing activity (Kobayashi et al, 2011). In addition to promoting the release of CCP110, TTBK2 also plays a role in the recruitment of intraflagellar transport (IFT) proteins and in this way contributes to extension of the ciliary axoneme (Goetz et al, 2012; Ye et al, 2014). Mutations in TTBK2 disrupt ciliogenesis and are associated with the development of spinocerebellar ataxia (Houlden et al, 2007; Bouskila et al, 2011; reviewed in Jackson, 2012).

C2CD3与远端附属蛋白CEP164对于激酶Tau微管蛋白激酶2（TTBK2）募集至中心粒的必要条件。TTBK2的募集促进母中心粒上负调控ciliogenesis的CCP110的释放（Goetz et al, 2012; Ye et al, 2014; Tsang et al, 2008; Spektor et al, 2007）。CCP110初始募集和/或稳定于母中心粒的方式依赖于KIF24；KIF24，一种类似驱动蛋白的蛋白，亦通过其微管解聚活性限制ciliogenesis（Kobayashi et al, 2011）。除了促进CCP110的释放，TTBK2还在内节间运输（IFT）蛋白的募集中发挥作用，从而有助于纤毛轴丝的延伸（Goetz et al, 2012; Ye et al, 2014）。TTBK2中的突变会破坏ciliogenesis并与其发展成脊髓小脑性共济失调相关（Houlden et al, 2007; Bouskila et al, 2011；详见Jackson, 2012年的综述）。