• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

多囊蛋白、粘着斑与细胞外基质相互作用。

Polycystins, focal adhesions and extracellular matrix interactions.

作者信息

Drummond Iain A

机构信息

Massachusetts General Hospital, MA, USA.

出版信息

Biochim Biophys Acta. 2011 Oct;1812(10):1322-6. doi: 10.1016/j.bbadis.2011.03.003. Epub 2011 Mar 9.

DOI:10.1016/j.bbadis.2011.03.003
PMID:21396443
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3132319/
Abstract

Polycystic kidney disease is the most common heritable disease in humans. In addition to epithelial cysts in the kidney, liver and pancreas, patients with autosomal dominant polycystic kidney disease (ADPKD) also suffer from abdominal hernia, intracranial aneurysm, gastrointestinal cysts, and cardiac valvular defects, conditions often associated with altered extracellular matrix production or integrity. Despite more than a decade of work on the principal ADPKD genes, PKD1 and PKD2, questions remain about the basis of cystic disease and the role of extracellular matrix in ADPKD pathology. This review explores the links between polycystins, focal adhesions, and extracellular matrix gene expression. These relationships suggest roles for polycystins in cell-matrix mechanosensory signaling that control matrix production and morphogenesis. This article is part of a Special Issue entitled: Polycystic Kidney Disease.

摘要

多囊肾病是人类最常见的遗传性疾病。除了肾脏、肝脏和胰腺出现上皮囊肿外,常染色体显性多囊肾病(ADPKD)患者还患有腹疝、颅内动脉瘤、胃肠道囊肿和心脏瓣膜缺损,这些病症通常与细胞外基质产生或完整性改变有关。尽管对主要的ADPKD基因PKD1和PKD2进行了十多年的研究,但关于囊性疾病的发病基础以及细胞外基质在ADPKD病理中的作用仍存在疑问。本综述探讨了多囊蛋白、黏着斑和细胞外基质基因表达之间的联系。这些关系表明多囊蛋白在控制基质产生和形态发生的细胞 - 基质机械感觉信号传导中发挥作用。本文是名为《多囊肾病》的特刊的一部分。

相似文献

1
Polycystins, focal adhesions and extracellular matrix interactions.多囊蛋白、粘着斑与细胞外基质相互作用。
Biochim Biophys Acta. 2011 Oct;1812(10):1322-6. doi: 10.1016/j.bbadis.2011.03.003. Epub 2011 Mar 9.
2
The ADPKD genes pkd1a/b and pkd2 regulate extracellular matrix formation.PKD1A/B 和 PKD2 基因调控细胞外基质的形成。
Dis Model Mech. 2010 May-Jun;3(5-6):354-65. doi: 10.1242/dmm.003194. Epub 2010 Mar 24.
3
Fibrosis and progression of autosomal dominant polycystic kidney disease (ADPKD).常染色体显性多囊肾病(ADPKD)的纤维化与进展
Biochim Biophys Acta. 2011 Oct;1812(10):1327-36. doi: 10.1016/j.bbadis.2011.06.012. Epub 2011 Jul 1.
4
Molecular basis of autosomal dominant polycystic kidney disease.常染色体显性多囊肾病的分子基础
Adv Anat Pathol. 2005 May;12(3):126-33. doi: 10.1097/01.pap.0000163959.29032.1f.
5
Cell-Autonomous Hedgehog Signaling Is Not Required for Cyst Formation in Autosomal Dominant Polycystic Kidney Disease.常染色体显性多囊肾病中,细胞自主 Hedgehog 信号传导对于囊肿形成不是必需的。
J Am Soc Nephrol. 2019 Nov;30(11):2103-2111. doi: 10.1681/ASN.2018121274. Epub 2019 Aug 26.
6
Scattered Deletion of PKD1 in Kidneys Causes a Cystic Snowball Effect and Recapitulates Polycystic Kidney Disease.肾脏中PKD1的散在缺失导致囊性雪球效应并重现多囊肾病。
J Am Soc Nephrol. 2015 Jun;26(6):1322-33. doi: 10.1681/ASN.2013080864. Epub 2014 Oct 31.
7
The role of the polycystins in kidney development.多囊蛋白在肾脏发育中的作用。
Pediatr Nephrol. 1999 Jun;13(5):454-9. doi: 10.1007/s004670050639.
8
Polycystins and renovascular mechanosensory transduction.多囊蛋白和肾血管机械感觉转导。
Nat Rev Nephrol. 2010 Sep;6(9):530-8. doi: 10.1038/nrneph.2010.97. Epub 2010 Jul 13.
9
Cyclin-Dependent Kinase 1 Activity Is a Driver of Cyst Growth in Polycystic Kidney Disease.周期蛋白依赖性激酶 1 活性是多囊肾病囊肿生长的驱动因素。
J Am Soc Nephrol. 2021 Jan;32(1):41-51. doi: 10.1681/ASN.2020040511. Epub 2020 Oct 12.
10
Somatic Mutations in Renal Cyst Epithelium in Autosomal Dominant Polycystic Kidney Disease.常染色体显性多囊肾病中肾囊肿上皮的体细胞突变。
J Am Soc Nephrol. 2018 Aug;29(8):2139-2156. doi: 10.1681/ASN.2017080878. Epub 2018 Jul 24.

引用本文的文献

1
Polycystins Expression in Astrocytic Gliomas.多囊蛋白在星形胶质细胞瘤中的表达
Biomedicines. 2025 Apr 5;13(4):884. doi: 10.3390/biomedicines13040884.
2
Advances and Challenges in Modeling Autosomal Dominant Polycystic Kidney Disease: A Focus on Kidney Organoids.常染色体显性多囊肾病建模的进展与挑战:聚焦肾类器官
Biomedicines. 2025 Feb 19;13(2):523. doi: 10.3390/biomedicines13020523.
3
Physiologic mechanisms underlying polycystic kidney disease.多囊肾病的生理机制。
Physiol Rev. 2025 Jul 1;105(3):1553-1607. doi: 10.1152/physrev.00018.2024. Epub 2025 Feb 12.
4
Activation of PIEZO1 Attenuates Kidney Cystogenesis In Vitro and Ex Vivo.PIEZO1的激活在体外和体内均能减轻肾囊肿的形成。
Kidney360. 2024 Nov 1;5(11):1601-1612. doi: 10.34067/KID.0000000598. Epub 2024 Oct 2.
5
Leucine-Rich Repeat in Polycystin-1 Suppresses Cystogenesis in a Zebrafish () Model of Autosomal-Dominant Polycystic Kidney Disease.富含亮氨酸重复序列的多囊蛋白-1抑制常染色体显性遗传多囊肾病斑马鱼模型中的囊肿生成。
Int J Mol Sci. 2024 Mar 1;25(5):2886. doi: 10.3390/ijms25052886.
6
Primary cilia and actin regulatory pathways in renal ciliopathies.肾脏纤毛病中的初级纤毛与肌动蛋白调节途径
Front Nephrol. 2024 Jan 16;3:1331847. doi: 10.3389/fneph.2023.1331847. eCollection 2023.
7
Prevalence of pericardial effusion in autosomal dominant polycystic kidney disease.常染色体显性多囊肾病中心包积液的患病率。
Clin Kidney J. 2023 Jul 20;16(11):2041-2047. doi: 10.1093/ckj/sfad181. eCollection 2023 Nov.
8
Primary cilia and hypoxia-associated signaling in developmental odontogenic cysts in relation to autosomal dominant polycystic kidney disease - A novel insight.原发性纤毛与发育性牙源性囊肿中与常染色体显性多囊肾病相关的缺氧信号传导——一项新见解
Heliyon. 2023 Jun 9;9(6):e17130. doi: 10.1016/j.heliyon.2023.e17130. eCollection 2023 Jun.
9
Urinary exosome proteomic profiling defines stage-specific rapid progression of autosomal dominant polycystic kidney disease and tolvaptan efficacy.尿液外泌体蛋白质组学分析确定了常染色体显性多囊肾病的阶段特异性快速进展及托伐普坦的疗效。
BBA Adv. 2021 May 13;1:100013. doi: 10.1016/j.bbadva.2021.100013. eCollection 2021.
10
Tracking N- and C-termini of C. elegans polycystin-1 reveals their distinct targeting requirements and functions in cilia and extracellular vesicles.追踪秀丽隐杆线虫多聚蛋白-1 的 N-和 C-末端揭示了它们在纤毛和细胞外囊泡中的不同靶向需求和功能。
PLoS Genet. 2022 Dec 27;18(12):e1010560. doi: 10.1371/journal.pgen.1010560. eCollection 2022 Dec.

本文引用的文献

1
Connective tissue alteration in abdominal wall hernia.腹壁疝的结缔组织改变。
Br J Surg. 2011 Feb;98(2):210-9. doi: 10.1002/bjs.7339.
2
Naturally occurring osmolytes modulate the nanomechanical properties of polycystic kidney disease domains.天然渗透物调节多囊肾病域的纳米力学特性。
J Biol Chem. 2010 Dec 3;285(49):38438-43. doi: 10.1074/jbc.M110.183913. Epub 2010 Oct 11.
3
Cyst formation in the PKD2 (1-703) transgenic rat precedes deregulation of proliferation-related pathways.PKD2(1-703)转基因大鼠中的囊肿形成先于增殖相关途径的失调。
BMC Nephrol. 2010 Sep 2;11:23. doi: 10.1186/1471-2369-11-23.
4
The ADPKD genes pkd1a/b and pkd2 regulate extracellular matrix formation.PKD1A/B 和 PKD2 基因调控细胞外基质的形成。
Dis Model Mech. 2010 May-Jun;3(5-6):354-65. doi: 10.1242/dmm.003194. Epub 2010 Mar 24.
5
Mechanistic insight into the function of the C-terminal PKD domain of the collagenolytic serine protease deseasin MCP-01 from deep sea Pseudoalteromonas sp. SM9913: binding of the PKD domain to collagen results in collagen swelling but does not unwind the collagen triple helix.深入了解深海假交替单胞菌 SM9913 胶原酶丝氨酸蛋白酶 deseasin MCP-01 的 C 端 PKD 结构域功能的机制:PKD 结构域与胶原的结合导致胶原肿胀,但不会使胶原三螺旋展开。
J Biol Chem. 2010 May 7;285(19):14285-91. doi: 10.1074/jbc.M109.087023. Epub 2010 Mar 5.
6
Cellular and molecular mechanisms of thoracic aortic aneurysms.胸主动脉瘤的细胞和分子机制。
Nat Rev Cardiol. 2009 Dec;6(12):771-86. doi: 10.1038/nrcardio.2009.191. Epub 2009 Nov 3.
7
Polycystin-1 and -2 dosage regulates pressure sensing.多囊蛋白-1和-2的剂量调节压力感知。
Cell. 2009 Oct 30;139(3):587-96. doi: 10.1016/j.cell.2009.08.045.
8
Naturally occurring mutations alter the stability of polycystin-1 polycystic kidney disease (PKD) domains.自然发生的突变会改变多囊蛋白-1多囊肾病(PKD)结构域的稳定性。
J Biol Chem. 2009 Nov 20;284(47):32942-9. doi: 10.1074/jbc.M109.021832. Epub 2009 Sep 15.
9
Mechanotransduction in development: a growing role for contractility.发育过程中的机械转导:收缩性的作用日益重要。
Nat Rev Mol Cell Biol. 2009 Jan;10(1):34-43. doi: 10.1038/nrm2592.
10
TRPCs, GPCRs and the Bayliss effect.瞬时受体电位通道、G蛋白偶联受体与贝利斯效应。
EMBO J. 2009 Jan 7;28(1):4-5. doi: 10.1038/emboj.2008.261.