关键词: PCM1 centriolar satellites centrosome ciliopathies membrane‐less organelle microtubule neurodevelopmental disorders primary cilium

来  源:   DOI:10.1111/febs.17194

Abstract:
Centriolar satellites are ubiquitous membrane-less organelles that play critical roles in numerous cellular and organismal processes. They were initially discovered through electron microscopy as cytoplasmic granules surrounding centrosomes in vertebrate cells. These structures remained enigmatic until the identification of pericentriolar material 1 protein (PCM1) as their molecular marker, which has enabled their in-depth characterization. Recently, centriolar satellites have come into the spotlight due to their links to developmental and neurodegenerative disorders. This review presents a comprehensive summary of the major advances in centriolar satellite biology, with a focus on studies that investigated their biology associated with the essential scaffolding protein PCM1. We begin by exploring the molecular, cellular, and biochemical properties of centriolar satellites, laying the groundwork for a deeper understanding of their functions and mechanisms at both cellular and organismal levels. We then examine the implications of their dysregulation in various diseases, particularly highlighting their emerging roles in neurodegenerative and developmental disorders, as revealed by organismal models of PCM1. We conclude by discussing the current state of knowledge and posing questions about the adaptable nature of these organelles, thereby setting the stage for future research.
摘要:
Centriolar卫星是普遍存在的无膜细胞器,在许多细胞和生物体过程中起关键作用。它们最初是通过电子显微镜发现的,是脊椎动物细胞中中心体周围的细胞质颗粒。这些结构仍然是神秘的,直到鉴定出四角材料1蛋白(PCM1)作为其分子标记,这使得他们能够进行深入的表征。最近,由于它们与发育和神经退行性疾病的联系,向心卫星已经成为人们关注的焦点。这篇综述全面总结了中心卫星生物学的主要进展,重点研究了与必需支架蛋白PCM1相关的生物学研究。我们首先探索分子,细胞,和中心卫星的生化特性,为更深入地了解它们在细胞和生物体层面的功能和机制奠定基础。然后,我们研究了它们在各种疾病中失调的含义,特别强调它们在神经退行性疾病和发育障碍中的新作用,PCM1的有机模型揭示了这一点。最后,我们讨论了当前的知识状况,并提出了有关这些细胞器的适应性的问题,从而为未来的研究奠定了基础。
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