Insights Into the Molecular Mechanisms of Polycystic Kidney Diseases.
Insights Into the Molecular Mechanisms of Polycystic Kidney Diseases.
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DOI：
10.3389/fphys.2021.693130
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发表时间：
2021
影响因子：
4
通讯作者：
Ilatovskaya DV
中科院分区：
文献类型：
--
作者：
Vasileva VY;Sultanova RF;Sudarikova AV;Ilatovskaya DV
常染色体显性遗传（AD）和常染色体隐性遗传（AR）多囊肾病（PKD）是严重的多系统遗传性疾病，其特征在于肾脏中充满液体的囊肿的形成和不受控制的生长，其扩散最终导致肾功能丧失。目前，还没有ARPKD的治疗方法，托伐普坦是FDA批准的唯一一种缓解ADPKD症状的药物。然而，托伐普坦对疾病进展只有适度的影响，长期使用与许多副作用有关。因此，仍然迫切需要更好地了解PKD发展背后的基本机制。本综述强调了目前对PKD发展（重点是ADPKD）的基本方面的了解，包括PC 1/PC 2途径和纤毛相关机制，与代谢相关的主要分子级联，线粒体生物能量学和影响其进展的全身反应（激素状态，生长因子水平，免疫系统和微生物组）。此外，我们还讨论了有关非药物治疗的新信息，如饮食限制，这可能会缓解PKD。
Autosomal dominant (AD) and autosomal recessive (AR) polycystic kidney diseases (PKD) are severe multisystem genetic disorders characterized with formation and uncontrolled growth of fluid-filled cysts in the kidney, the spread of which eventually leads to the loss of renal function. Currently, there are no treatments for ARPKD, and tolvaptan is the only FDA-approved drug that alleviates the symptoms of ADPKD. However, tolvaptan has only a modest effect on disease progression, and its long-term use is associated with many side effects. Therefore, there is still a pressing need to better understand the fundamental mechanisms behind PKD development. This review highlights current knowledge about the fundamental aspects of PKD development (with a focus on ADPKD) including the PC1/PC2 pathways and cilia-associated mechanisms, major molecular cascades related to metabolism, mitochondrial bioenergetics, and systemic responses (hormonal status, levels of growth factors, immune system, and microbiome) that affect its progression. In addition, we discuss new information regarding non-pharmacological therapies, such as dietary restrictions, which can potentially alleviate PKD.
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作者：
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影响因子：
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