The major hallmark of Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the formation of many fluid-filled cysts in the kidneys, which ultimately impairs the normal renal structure and function, leading to end-stage renal disease (ESRD). A large body of evidence suggests that injury-repair mechanisms are part of ADPKD progression. Once cysts have been formed, proliferation and fluid secretion contribute to the cyst size increase, which eventually causes stress on the surrounding tissue resulting in local injury and fibrosis. In addition, renal injury can cause or accelerate cyst formation. In this review, we will describe the various mechanisms activated during renal injury and tissue repair and show how they largely overlap with the molecular mechanisms activated during PKD progression. In particular, we will discuss molecular mechanisms such as proliferation, inflammation, cell differentiation, cytokines and growth factors secretion, which are activated following the renal injury to allow the remodelling of the tissue and a proper organ repair. We will also underline how, in a context of PKD-related gene mutations, aberrant or chronic activation of these developmental pathways and repair/remodelling mechanisms results in exacerbation of the disease.

中文翻译：

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参与损伤修复和 ADPKD 进展的分子途径。

常染色体显性多囊肾病 (ADPKD) 的主要标志是肾脏中形成许多充满液体的囊肿，最终损害正常的肾脏结构和功能，导致终末期肾病 (ESRD)。大量证据表明，损伤修复机制是 ADPKD 进展的一部分。一旦囊肿形成，增殖和液体分泌会导致囊肿大小增加，最终对周围组织造成压力，导致局部损伤和纤维化。此外，肾损伤可导致或加速囊肿形成。在这篇综述中，我们将描述在肾损伤和组织修复过程中激活的各种机制，并展示它们如何与 PKD 进展过程中激活的分子机制在很大程度上重叠。特别是，我们将讨论分子机制，如增殖、炎症、细胞分化、细胞因子和生长因子分泌，这些在肾损伤后被激活，以允许组织重塑和适当的器官修复。我们还将强调在 PKD 相关基因突变的背景下，这些发育途径和修复/重塑机制的异常或慢性激活如何导致疾病恶化。