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检查crispant 和突变斑马鱼人类骨骼疾病模型的颅面变异。

Examining craniofacial variation among crispant and mutant zebrafish models of human skeletal diseases.

机构信息

Department of Biology, Rhodes College, Tennessee, Memphis, USA.

Center for Developmental Biology and Regenerative Medicine, Seattle Children's Research Institute, Seattle, Washington, USA.

出版信息

J Anat. 2023 Jul;243(1):66-77. doi: 10.1111/joa.13847. Epub 2023 Mar 1.

Abstract

Genetic diseases affecting the skeletal system present with a wide range of symptoms that make diagnosis and treatment difficult. Genome-wide association and sequencing studies have identified genes linked to human skeletal diseases. Gene editing of zebrafish models allows researchers to further examine the link between genotype and phenotype, with the long-term goal of improving diagnosis and treatment. While current automated tools enable rapid and in-depth phenotyping of the axial skeleton, characterizing the effects of mutations on the craniofacial skeleton has been more challenging. The objective of this study was to evaluate a semi-automated screening tool can be used to quantify craniofacial variations in zebrafish models using four genes that have been associated with human skeletal diseases (meox1, plod2, sost, and wnt16) as test cases. We used traditional landmarks to ground truth our dataset and pseudolandmarks to quantify variation across the 3D cranial skeleton between the groups (somatic crispant, germline mutant, and control fish). The proposed pipeline identified variation between the crispant or mutant fish and control fish for four genes. Variation in phenotypes parallel human craniofacial symptoms for two of the four genes tested. This study demonstrates the potential as well as the limitations of our pipeline as a screening tool to examine multi-dimensional phenotypes associated with the zebrafish craniofacial skeleton.

摘要

影响骨骼系统的遗传疾病表现出多种症状,这使得诊断和治疗变得困难。全基因组关联和测序研究已经确定了与人类骨骼疾病相关的基因。斑马鱼模型的基因编辑使研究人员能够进一步研究基因型和表型之间的联系,长期目标是改善诊断和治疗。虽然当前的自动化工具能够快速深入地表征轴骨骼的表型,但表征突变对颅面骨骼的影响更具挑战性。本研究的目的是评估一种半自动筛选工具,该工具可用于使用与人类骨骼疾病相关的四个基因(meox1、plod2、sost 和 wnt16)作为测试案例来量化斑马鱼模型中的颅面变化。我们使用传统的地标来为我们的数据集提供基准,并使用伪地标来量化组间(躯体crispant、生殖系突变体和对照鱼)的 3D 颅骨变异。所提出的流水线识别了crispant 或突变鱼与对照鱼之间的四个基因的变异。在测试的四个基因中,有两个基因的表型变化与人类颅面症状平行。这项研究表明了我们的流水线作为一种筛选工具,用于检查与斑马鱼颅面骨骼相关的多维表型的潜力和局限性。

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