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纤毛病基因Ick在成年小鼠细胞增殖区域的表达增强。

Enriched expression of the ciliopathy gene Ick in cell proliferating regions of adult mice.

作者信息

Tsutsumi Ryotaro, Chaya Taro, Furukawa Takahisa

机构信息

Laboratory for Molecular and Developmental Biology, Institute for Protein Research, Osaka University, 3-2 Yamadaoka, Suita, Osaka, 565-0871, Japan.

Laboratory for Molecular and Developmental Biology, Institute for Protein Research, Osaka University, 3-2 Yamadaoka, Suita, Osaka, 565-0871, Japan.

出版信息

Gene Expr Patterns. 2018 Sep;29:18-23. doi: 10.1016/j.gep.2018.04.005. Epub 2018 Apr 7.

DOI:10.1016/j.gep.2018.04.005
PMID:29635032
Abstract

Cilia are essential for sensory and motile functions across species. In humans, ciliary dysfunction causes "ciliopathies", which show severe developmental abnormalities in various tissues. Several missense mutations in intestinal cell kinase (ICK) gene lead to endocrine-cerebro-osteodysplasia syndrome or short rib-polydactyly syndrome, lethal recessive developmental ciliopathies. We and others previously reported that Ick-deficient mice exhibit neonatal lethality with developmental defects. Mechanistically, Ick regulates intraflagellar transport and cilia length at ciliary tips. Although Ick plays important roles during mammalian development, roles of Ick at the adult stage are poorly understood. In the current study, we investigated the Ick gene expression in adult mouse tissues. RT-PCR analysis showed that Ick is ubiquitously expressed, with enrichment in the retina, brain, lung, intestine, and reproductive system. In the adult brain, we found that Ick expression is enriched in the walls of the lateral ventricle, in the rostral migratory stream of the olfactory bulb, and in the subgranular zone of the hippocampal dentate gyrus by in situ hybridization analysis. We also observed that Ick staining pattern is similar to pachytene spermatocyte to spermatid markers in the mature testis and to an intestinal stem cell marker in the adult small intestine. These results suggest that Ick is expressed in proliferating regions in the adult mouse brain, testis, and intestine.

摘要

纤毛对于跨物种的感觉和运动功能至关重要。在人类中,纤毛功能障碍会导致“纤毛病”,其在各种组织中表现出严重的发育异常。肠道细胞激酶(ICK)基因中的几个错义突变会导致内分泌-脑-骨发育异常综合征或短肋多指综合征,这是致命的隐性发育性纤毛病。我们和其他人之前报道过,Ick基因缺陷的小鼠表现出具有发育缺陷的新生儿致死性。从机制上讲,Ick在纤毛尖端调节鞭毛内运输和纤毛长度。尽管Ick在哺乳动物发育过程中发挥重要作用,但对其在成年阶段的作用了解甚少。在本研究中,我们调查了成年小鼠组织中Ick基因的表达。逆转录聚合酶链反应(RT-PCR)分析表明,Ick在全身广泛表达,在视网膜、脑、肺、肠道和生殖系统中表达丰富。在成年大脑中,通过原位杂交分析,我们发现Ick在侧脑室壁、嗅球的吻侧迁移流以及海马齿状回的颗粒下区表达丰富。我们还观察到,在成熟睾丸中,Ick的染色模式与粗线期精母细胞到精子细胞的标记物相似,在成年小肠中与肠道干细胞标记物相似。这些结果表明,Ick在成年小鼠的脑、睾丸和肠道的增殖区域表达。

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