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首页> 外文期刊>Current opinion in gastroenterology >Cholangiociliopathies: genetics, molecular mechanisms and potential therapies.
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Cholangiociliopathies: genetics, molecular mechanisms and potential therapies.

机译：胆管疾病：遗传学，分子机制和潜在疗法。

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PURPOSE OF REVIEW: The present review summarizes recent knowledge on polycystic liver diseases (PCLDs), mechanisms of hepatic cystogenesis and potential therapies for these conditions. RECENT FINDINGS: PCLD may be classified as cholangiociliopathies. In PCLD associated with polycystic kidney disease, cell proliferation is one of the major mechanisms of cystogenesis, whereas in isolated PCLD (autosomal dominant polycystic liver disease), disrupted cell adhesion may be more important in cyst progression. In cystic cholangiocytes, overexpression of ion transporters and water channels facilitates fluid secretion into the cystic lumen, and growth factors, estrogens and cytokines promote cholangiocyte proliferation. With age, cholangiocytes lining liver cysts acquire features of mesenchymal cells contributing to hepatic fibrocystogenesis. A novel mechanism of liver cyst expansion in PCLD involves microRNA regulatory pathways. Hyperproliferation of cystic cholangiocytes is linked to abnormalities in cell cycle progression and microRNA expression. Decreased levels of miR-15a are coupled to upregulation of its target--the cell cycle regulator, Cdc25A. Cholangiocyte cilia in liver cysts are structurally abnormal. Somatostatin analogues and sirolimus reduce liver cyst volume in PCLD patients. SUMMARY: Clarification of molecular mechanisms of hepatic cystogenesis provides an opportunity for the development of targeted therapeutic options in PCLD.
机译：审查的目的：本综述总结了有关多囊性肝病（PCLDs），肝囊肿发生机制和针对这些情况的潜在疗法的最新知识。最近发现：PCLD可归类为胆管疾病。在与多囊性肾病相关的PCLD中，细胞增殖是囊肿发生的主要机制之一，而在孤立的PCLD（常染色体显性多囊性肝病）中，细胞黏附破坏在囊肿进展中可能更为重要。在囊性胆管细胞中，离子转运蛋白和水通道的过表达促进液体分泌进入囊性管腔，而生长因子，雌激素和细胞因子促进胆管细胞增殖。随着年龄的增长，位于肝囊肿内的胆管细胞会获得间充质细胞的特征，从而促进肝纤维囊肿的形成。 PCLD中肝囊肿扩展的新机制涉及microRNA调节途径。囊性胆管细胞的过度增殖与细胞周期进程和microRNA表达异常有关。 miR-15a水平的降低与其靶标-细胞周期调节剂Cdc25A的上调相关。肝囊肿中的胆​​管细胞纤毛结构异常。生长抑素类似物和西罗莫司可减少PCLD患者的肝囊肿体积。摘要：阐明肝囊肿形成的分子机制为PCLD中靶向治疗选择的发展提供了机会。

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