Polycystic kidneys frequently associate with mutations in individual components of cilia, basal bodies or centriolar satellites that perturb complex protein networks. In this review, we focus on the RNA-binding protein Bicaudal-C1 (BICC1) which was found mutated in renal cystic dysplasia, and on its interactions with the ankyrin repeat and sterile α motif (SAM)-containing proteins ANKS3 and ANKS6 and associated kinases and their partially overlapping ciliopathy phenotypes. After reviewing BICC1 homologs in model organisms and their functions in mRNA and cell metabolism during development and in renal tubules, we discuss recent insights from cell-based assays and from structure analysis of the SAM domains, and how SAM domain oligomerization might influence multivalent higher order complexes that are implicated in ciliary signal transduction.

中文翻译：

---

RNA结合蛋白Bicaudal-C1和相互作用因子在囊性肾脏疾病中的作用。

多囊肾经常与纤毛，基底小体或中心粒卫星单个成分的突变有关，这些突变扰乱了复杂的蛋白质网络。在这篇综述中，我们关注于肾囊性不典型增生中突变的RNA结合蛋白Bicaudal-C1（BICC1），及其与锚蛋白重复序列​​和含有无菌α基序（SAM）的蛋白ANKS3和ANKS6以及相关蛋白的相互作用激酶及其部分重叠的睫状体疾病表型。在回顾了模型生物中的BICC1同源物及其在发育过程中和在肾小管中的mRNA和细胞代谢功能之后，我们讨论了基于细胞的测定和SAM结构域结构分析的最新见解，以及SAM结构域寡聚化如何影响多价高阶反应与纤毛信号转导有关的复合物。