Vascular polycystin proteins in health and disease.
Vascular polycystin proteins in health and disease.
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健康和疾病中的血管多囊蛋白。
DOI：
10.1111/micc.12834
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发表时间：
2023
期刊：
影响因子：
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通讯作者：
Jaggar,JonathanH
中科院分区：
文献类型：
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作者：
Mbiakop,UlrichC;Jaggar,JonathanH
PKD 1（多囊蛋白1）和PKD 2（多囊蛋白2）在多种不同的细胞类型中表达，包括动脉平滑肌和内皮细胞。PKD 1是一种跨膜结构域蛋白，具有大的细胞外N末端，被认为是机械传感器和受体。PKD 2是瞬时受体电位（TRP）通道超家族的成员，也称为TRPP 1。编码PKD 1和PKD 2的基因突变会导致常染色体显性多囊肾病（ADPKD）。ADPKD是人类最常见的单基因疾病之一，与肾外和血管并发症（包括高血压）相关。最近的研究揭示了PKD 1和PKD 2在动脉平滑肌和内皮细胞中的激活和生理功能的机制。还发现在ADPKD和高血压期间，PKD功能在血管系统中改变。我们将总结这项工作，并讨论这一研究领域的未来可能性。
PKD1 (polycystin 1) and PKD2 (polycystin 2) are expressed in a variety of different cell types, including arterial smooth muscle and endothelial cells. PKD1 is a transmembrane domain protein with a large extracellular N‐terminus that is proposed to act as a mechanosensor and receptor. PKD2 is a member of the transient receptor potential (TRP) channel superfamily which is also termed TRPP1. Mutations in the genes which encode PKD1 and PKD2 lead to autosomal dominant polycystic kidney disease (ADPKD). ADPKD is one of the most prevalent monogenic disorders in humans and is associated with extrarenal and vascular complications, including hypertension. Recent studies have uncovered mechanisms of activation and physiological functions of PKD1 and PKD2 in arterial smooth muscle and endothelial cells. It has also been found that PKD function is altered in the vasculature during ADPKD and hypertension. We will summarize this work and discuss future possibilities for this area of research.