Small GTPases are known to have pivotal roles in intracellular trafficking, and several members of the small GTPases superfamily such as Rab10 [1,2•], Rab11 [3-5], Rab34 [6•,7], Rab8 [3,8], Rab23 [9-12], RSG1 [13-15], Arl13b [16-22], and Arl6 [22,23] were recently reported to mediate primary cilia function and/or Hh signalling. Although these functions are implicated in diseases such as ciliopathies, the molecular basis underlying how these small GTPases mediate primary cilia-dependent Hh signalling and pathogenesis of ciliopathies warrants further investigations. Notably, Rab23 and Arl13b have been implicated in ciliopathy-associated human diseases and could regulate Hh signalling cascade in multifaceted manners. This review thus specifically discuss the roles of Rab23 and Arl13b in primary cilia of mammalian systems, their cilia-dependent and cilia-independent modulation of hedgehog signalling pathways and their implications in Carpenter Syndrome and Joubert Syndrome respectively.

中文翻译：

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刺猬信号中的小GTP酶：对Rab23介导的和Arl13b介导的纤毛病的发病机理的新见解。

已知小GTPases在细胞内运输中起关键作用，并且小GTPases超家族的几个成员，如Rab10 [1,2•]，Rab11 [3-5]，Rab34 [6•，7]，Rab8 [3,8] ]，Rab23 [9-12]，RSG1 [13-15]，Arl13b [16-22]和Arl6 [22,23]最近被报道介导初级纤毛功能和/或Hh信号传导。尽管这些功能牵涉到诸如纤毛虫病的疾病中，但这些小GTP酶如何介导纤毛依赖性Hh信号原发和纤毛虫病的发病机理的分子基础值得进一步研究。值得注意的是，Rab23和Arl13b已经与纤毛病相关的人类疾病有关，并且可以以多种方式调节Hh信号级联。因此，本综述专门讨论了Rab23和Arl13b在哺乳动物系统的初级纤毛中的作用，