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在遗传性多系统疾病的机制和药物研究中,斑马鱼模型中过早的椎体矿化的意义。

Significance of Premature Vertebral Mineralization in Zebrafish Models in Mechanistic and Pharmaceutical Research on Hereditary Multisystem Diseases.

机构信息

Center for Medical Genetics, Ghent University Hospital, 9000 Ghent, Belgium.

Department of Biomolecular Medicine, Ghent University, 9000 Ghent, Belgium.

出版信息

Biomolecules. 2023 Nov 6;13(11):1621. doi: 10.3390/biom13111621.

Abstract

Zebrafish are increasingly becoming an important model organism for studying the pathophysiological mechanisms of human diseases and investigating how these mechanisms can be effectively targeted using compounds that may open avenues to novel treatments for patients. The zebrafish skeleton has been particularly instrumental in modeling bone diseases as-contrary to other model organisms-the lower load on the skeleton of an aquatic animal enables mutants to survive to early adulthood. In this respect, the axial skeletons of zebrafish have been a good read-out for congenital spinal deformities such as scoliosis and degenerative disorders such as osteoporosis and osteoarthritis, in which aberrant mineralization in humans is reflected in the respective zebrafish models. Interestingly, there have been several reports of hereditary multisystemic diseases that do not affect the vertebral column in human patients, while the corresponding zebrafish models systematically show anomalies in mineralization and morphology of the spine as their leading or, in some cases, only phenotype. In this review, we describe such examples, highlighting the underlying mechanisms, the already-used or potential power of these models to help us understand and amend the mineralization process, and the outstanding questions on how and why this specific axial type of aberrant mineralization occurs in these disease models.

摘要

斑马鱼正日益成为研究人类疾病病理生理机制的重要模式生物,并研究如何使用可能为患者开辟新治疗途径的化合物来有效靶向这些机制。斑马鱼骨骼在模拟骨骼疾病方面特别有用,因为与其他模式生物相比,水生动物骨骼的负荷较低,使突变体能够存活到成年早期。在这方面,斑马鱼的轴向骨骼是先天性脊柱侧凸等脊柱畸形和骨质疏松症和骨关节炎等退行性疾病的良好指标,在这些疾病中,人类的异常矿化反映在各自的斑马鱼模型中。有趣的是,有几例遗传性多系统疾病的报告称,人类患者的脊柱不受影响,而相应的斑马鱼模型则系统地显示出脊柱矿化和形态的异常,作为其主要的(在某些情况下,是唯一的)表型。在这篇综述中,我们描述了这些例子,强调了潜在的机制,以及这些模型已经使用或潜在的力量,以帮助我们理解和修正矿化过程,以及关于这种特定的轴向异常矿化如何以及为什么会在这些疾病模型中发生的悬而未决的问题。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3066/10669475/f439b4ad6a1b/biomolecules-13-01621-g003.jpg

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