The Players: Cells Involved in Glomerular Disease
The Players: Cells Involved in Glomerular Disease
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DOI：
10.2215/cjn.13791215
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发表时间：
2016-09-01
影响因子：
9.8
通讯作者：
Hutton, Holly L.
中科院分区：
文献类型：
--
作者：
Kitching, A. Richard;Hutton, Holly L.
肾小球疾病是常见且重要的疾病。它们可由影响肾脏的全身性炎症或代谢性疾病引起。另外，它们主要是由局部肾小球异常引起的，包括遗传性疾病。内在肾小球细胞和白细胞对健康的肾小球和疾病中的肾小球失调都至关重要。肾小球内的系膜细胞、内皮细胞、足细胞和壁上皮细胞都发挥着独特而特殊的作用。虽然一种特定的疾病通常主要影响一种特定的细胞类型，但细胞之间的密切关系和相互依存的功能和相互作用意味着，即使影响一种细胞类型的疾病也通常间接影响其他细胞类型。除了那些肾小球固有的细胞外，白细胞在健康时巡视肾小球，并在疾病时介导损伤。存在不同的白细胞类型和亚群，其中一些在个体肾小球疾病中以不同的方式参与。先天免疫和适应性免疫系统的细胞很重要，它们指导全身免疫和炎症反应，局部介导损伤，并可能抑制炎症和促进修复。新的遗传和分子技术的出现，以及新的疾病模型意味着我们更好地了解肾小球的基本生物学和肾小球疾病的发病机制。这种认识将导致更好的诊断技术、生物标志物和预后、疾病严重程度和复发的预测因子。有了这些知识，未来有希望有更好的治疗方法，旨在阻止损伤和纤维化的途径，或中断导致严重和进行性肾小球疾病的个体疾病的潜在病理生理学。
Glomerular diseases are common and important. They can arise from systemic inflammatory or metabolic diseases that affect the kidney. Alternately, they are caused primarily by local glomerular abnormalities, including genetic diseases. Both intrinsic glomerular cells and leukocytes are critical to the healthy glomerulus and to glomerular dysregulation in disease. Mesangial cells, endothelial cells, podocytes, and parietal epithelial cells within the glomerulus all play unique and specialized roles. Although a specific disease often primarily affects a particular cell type, the close proximity, and interdependent functions and interactions between cells mean that even diseases affecting one cell type usually indirectly influence others. In addition to those cells intrinsic to the glomerulus, leukocytes patrol the glomerulus in health and mediate injury in disease. Distinct leukocyte types and subsets are present, with some being involved in different ways in an individual glomerular disease. Cells of the innate and adaptive immune systems are important, directing systemic immune and inflammatory responses, locally mediating injury, and potentially dampening inflammation and facilitating repair. The advent of new genetic and molecular techniques, and new disease models means that we better understand both the basic biology of the glomerulus and the pathogenesis of glomerular disease. This understanding should lead to better diagnostic techniques, biomarkers, and predictors of prognosis, disease severity, and relapse. With this knowledge comes the promise of better therapies in the future, directed toward halting pathways of injury and fibrosis, or interrupting the underlying pathophysiology of the individual diseases that lead to significant and progressive glomerular disease.