首页|Tectonic蛋白家族的分子功能与相关疾病的研究进展

Tectonic蛋白家族的分子功能与相关疾病的研究进展

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Tectonic(TCTN),源于古希腊语,意为构造,表明该蛋白对细胞功能的重要构建作用.在人类和纤毛研究的模式生物中,该家族蛋白成员一般有1~3个.TCTN蛋白家族定位在细胞纤毛的基部过渡区,构建了纤毛基部"门"结构,负责胞质与纤毛间的物质分选,调控纤毛功能.此外,其还可通过调控Hedgehog信号通路和细胞周期等,介导细胞发育.TCTN家族基因突变常导致纤毛病发生,并且异常表达与癌症的进展相关,可作为肿瘤治疗的潜在靶标.本文主要对TCTN蛋白家族的分子功能和相关疾病进行分析与总结,为理解、预防、治疗TCTN突变导致的纤毛病和相关肿瘤提供理论参考及指导.
Molecular function and related disease of the Tectonic protein family
Tectonic(TCTN),derived from ancient Greek,means construction,indicating its important role in constructing cellular function.There are generally 1~3 protein members of this family in the model organisms for ciliary research including human.The TCTN protein family is located at the ciliary base called the transition zone,constructing a ciliary gate responsible for components sorting between the cytoplasm and cilia,and regulating the ciliary functions.In addition,cellular development could also be regulated by TCTN proteins via the Hedgehog signaling pathway and cell cycle.Mutations in TCTN family genes often lead to ciliopathies,and abnormal expression of TCTN is associated with the progression of cancer,making it a potential target for tumor treatment.In this review,we discuss and summarize the molecular functions and related diseases of the TCTN family,providing theoretical reference and guidance for understanding,preventing,and treating ciliopathies and related tumors caused by TCTN mutations.

Tectonicciliaciliary transition zoneciliopathiesHedgehog signaling pathwaycell cycle

蒋林、郁慧敏、徐闯、李亚、王亮

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江苏师范大学生命学学院,徐州 221116

Tectonic 纤毛 纤毛过渡区 纤毛病 Hedgehog信号通路 细胞周期

江苏省自然科学基金面上项目徐州市重点研发计划(社会发展)项目江苏高校优势学科建设工程资助项目(PAPD)

BK2023-1351KC-23300

2024

生命科学
国家自然科学基金委员会生命科学部 中国科学院生命科学与生物技术局 中国科学院生命科学和医学学部 中国科学院上海生命科学研究院

生命科学

CSTPCD
影响因子:0.542
ISSN:1004-0374
年,卷(期):2024.36(6)