睫状蛋白 ARL13B 抑制小鼠发育性肾囊肿发生。
Ciliary ARL13B inhibits developmental kidney cystogenesis in mouse.
机构信息
Department of Human Genetics, Emory University School of Medicine, 615 Michael Street, Suite 301, Atlanta, GA, 30322, USA.
Department of Human Genetics, Emory University School of Medicine, 615 Michael Street, Suite 301, Atlanta, GA, 30322, USA; Department of Biology, Brown University, Providence, RI, 02912, USA.
出版信息
Dev Biol. 2023 Aug;500:1-9. doi: 10.1016/j.ydbio.2023.05.004. Epub 2023 May 18.
ARL13B is a small GTPase enriched in cilia. Deletion of Arl13b in mouse kidney results in renal cysts and an associated absence of primary cilia. Similarly, ablation of cilia leads to kidney cysts. To investigate whether ARL13B functions from within cilia to direct kidney development, we examined kidneys of mice expressing an engineered cilia-excluded ARL13B variant, ARL13B. These mice retained renal cilia and developed cystic kidneys. Because ARL13B functions as a guanine nucleotide exchange factor (GEF) for ARL3, we examined kidneys of mice expressing an ARL13B variant that lacks ARL3 GEF activity, ARL13B. We found normal kidney development with no evidence of cysts in these mice. Taken together, our results show that ARL13B functions within cilia to inhibit renal cystogenesis during mouse development, and that this function does not depend on its role as a GEF for ARL3.
ARL13B 是一种富含纤毛的小 GTPase。在小鼠肾脏中敲除 Arl13b 会导致肾囊肿和初级纤毛缺失。同样，纤毛的缺失也会导致肾脏囊肿。为了研究 ARL13B 是否从纤毛内发挥作用来指导肾脏发育，我们检查了表达工程化纤毛排除 ARL13B 变体的小鼠的肾脏，ARL13B。这些小鼠保留了肾脏纤毛并发育出囊性肾脏。由于 ARL13B 作为 ARL3 的鸟嘌呤核苷酸交换因子（GEF）发挥作用，我们检查了表达缺乏 ARL3 GEF 活性的 ARL13B 变体的小鼠的肾脏，ARL13B。我们发现这些小鼠的肾脏发育正常，没有囊肿的证据。总之，我们的结果表明，ARL13B 在纤毛内发挥作用，抑制小鼠发育过程中的肾脏囊肿形成，而这一功能不依赖于其作为 ARL3 的 GEF 的作用。
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