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骨骼疾病的斑马鱼发育模型

Zebrafish Developmental Models of Skeletal Diseases.

作者信息

Luderman Lauryn N, Unlu Gokhan, Knapik Ela W

机构信息

Vanderbilt University Medical Center, Nashville, TN, United States; Vanderbilt Brain Institute, Vanderbilt University, Nashville, TN, United States; Vanderbilt Genetics Institute, Vanderbilt University, Nashville, TN, United States.

Vanderbilt University Medical Center, Nashville, TN, United States; Vanderbilt Genetics Institute, Vanderbilt University, Nashville, TN, United States; Vanderbilt University, Nashville, TN, United States.

出版信息

Curr Top Dev Biol. 2017;124:81-124. doi: 10.1016/bs.ctdb.2016.11.004. Epub 2017 Jan 4.

Abstract

The zebrafish skeleton shares many similarities with human and other vertebrate skeletons. Over the past years, work in zebrafish has provided an extensive understanding of the basic developmental mechanisms and cellular pathways directing skeletal development and homeostasis. This review will focus on the cell biology of cartilage and bone and how the basic cellular processes within chondrocytes and osteocytes function to assemble the structural frame of a vertebrate body. We will discuss fundamental functions of skeletal cells in production and secretion of extracellular matrix and cellular activities leading to differentiation of progenitors to mature cells that make up the skeleton. We highlight important examples where findings in zebrafish provided direction for the search for genes causing human skeletal defects and also how zebrafish research has proven important for validating candidate human disease genes. The work we cover here illustrates utility of zebrafish in unraveling molecular mechanisms of cellular functions necessary to form and maintain a healthy skeleton.

摘要

斑马鱼的骨骼与人类及其他脊椎动物的骨骼有许多相似之处。在过去几年里,对斑马鱼的研究让我们对指导骨骼发育和稳态的基本发育机制及细胞途径有了广泛的了解。本综述将聚焦于软骨和骨骼的细胞生物学,以及软骨细胞和骨细胞内的基本细胞过程如何发挥作用来构建脊椎动物身体的结构框架。我们将讨论骨骼细胞在细胞外基质产生和分泌中的基本功能,以及导致祖细胞分化为构成骨骼的成熟细胞的细胞活动。我们重点介绍一些重要的例子,其中斑马鱼的研究成果为寻找导致人类骨骼缺陷的基因提供了方向,以及斑马鱼研究如何证明对验证人类疾病候选基因很重要。我们在此涵盖的工作说明了斑马鱼在揭示形成和维持健康骨骼所需的细胞功能分子机制方面的效用。

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