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Rmrp Mutation Disrupts Chondrogenesis and Bone Ossification in Zebrafish Model of Cartilage-Hair Hypoplasia via Enhanced Wnt/β-Catenin Signaling
Sun,Xianding1; Zhang,Ruobin1; Liu,Mi1; Chen,Hangang1; Chen,Liang1; Luo,Fengtao1; Zhang,Dali1; Huang,Junlan1; Li,Fangfang1; Ni,Zhenhong1; Qi,Huabing1; Su,Nan1; Jin,Min1; Yang,Jing1; Tan,Qiaoyan1; Du,Xiaolan1; Chen,Bo1; Huang,Haiyang1; Chen,Shuai1; Yin,Liangjun2; Xu,Xiaoling3; Deng,Chuxia3; Luo,Lingfei4; Xie,Yangli1; Chen,Lin1
2019-11-01
Source PublicationJournal of Bone and Mineral Research
ISSN0884-0431
Volume34Issue:11Pages:2101-2116
AbstractCartilage-hair hypoplasia (CHH) is an autosomal recessive metaphyseal chondrodysplasia characterized by bone dysplasia and many other highly variable features. The gene responsible for CHH is the RNA component of the mitochondrial RNA-processing endoribonuclease (RMRP) gene. Currently, the pathogenesis of osteochondrodysplasia and extraskeletal manifestations in CHH patients remains incompletely understood; in addition, there are no viable animal models for CHH. We generated an rmrp KO zebrafish model to study the developmental mechanisms of CHH. We found that rmrp is required for the patterning and shaping of pharyngeal arches. Rmrp mutation inhibits the intramembranous ossification of skull bones and promotes vertebrae ossification. The abnormalities of endochondral bone ossification are variable, depending on the degree of dysregulated chondrogenesis. Moreover, rmrp mutation inhibits cell proliferation and promotes apoptosis through dysregulating the expressions of cell-cycle- and apoptosis-related genes. We also demonstrate that rmrp mutation upregulates canonical Wnt/β-catenin signaling; the pharmacological inhibition of Wnt/β-catenin could partially alleviate the chondrodysplasia and increased vertebrae mineralization in rmrp mutants. Our study, by establishing a novel zebrafish model for CHH, partially reveals the underlying mechanism of CHH, hence deepening our understanding of the role of rmrp in skeleton development.
KeywordCARTILAGE-HAIR HYPOPLASIA RMRP SKELETAL DEVELOPMENT WNT/Β-CATENIN ZEBRAFISH
DOI10.1002/jbmr.3820
URLView the original
Language英语
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Cited Times [WOS]:2   [WOS Record]     [Related Records in WOS]
Document TypeJournal article
CollectionFaculty of Health Sciences
Corresponding AuthorChen,Lin
Affiliation1.Laboratory of Wound Repair and Rehabilitation,State Key Laboratory of Trauma,Burns and Combined Injury,Trauma Center,Research Institute of Surgery,Daping Hospital,Army Medical University,Chongqing,400042,China
2.Department of Orthopedic Surgery,The Second Affiliated Hospital,Chongqing Medical University,Chongqing,400010,China
3.Faculty of Health Sciences,University of Macau,Macao
4.Key Laboratory of Freshwater Fish Reproduction and Development,Ministry of Education,Laboratory of Molecular Developmental Biology,School of Life Sciences,Southwest University,Chongqing,400715,China
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Sun,Xianding,Zhang,Ruobin,Liu,Mi,et al. Rmrp Mutation Disrupts Chondrogenesis and Bone Ossification in Zebrafish Model of Cartilage-Hair Hypoplasia via Enhanced Wnt/β-Catenin Signaling[J]. Journal of Bone and Mineral Research,2019,34(11):2101-2116.
APA Sun,Xianding.,Zhang,Ruobin.,Liu,Mi.,Chen,Hangang.,Chen,Liang.,...&Chen,Lin.(2019).Rmrp Mutation Disrupts Chondrogenesis and Bone Ossification in Zebrafish Model of Cartilage-Hair Hypoplasia via Enhanced Wnt/β-Catenin Signaling.Journal of Bone and Mineral Research,34(11),2101-2116.
MLA Sun,Xianding,et al."Rmrp Mutation Disrupts Chondrogenesis and Bone Ossification in Zebrafish Model of Cartilage-Hair Hypoplasia via Enhanced Wnt/β-Catenin Signaling".Journal of Bone and Mineral Research 34.11(2019):2101-2116.
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