Explanipedia

Comprehensive three-dimensional microCT and signaling analysis reveal the teratogenic eﬀect of 2,3,7,8-tetrachlorodibenzo-p-dioxin on craniofacial bone development in mice Open

Xiaoming Wang, Chunlin Qin, Dou Li, Xinran Xu, Xiaojing Pan , et al. · 2025

Exposure to 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD) in utero can result in osteogenic defect during palatogenesis, but the effects on other craniofacial bones and underlying mechanisms remain to be characterized. By treating pregnant mi…

FAM20A is a golgi-localized Type II transmembrane protein Open

Mohd Faizan Siddiqui, Jiahe Li, Shuzhen Wang, Hua Zhang, Chunlin Qin , et al. · 2024

Constitutive expression of spliced X-box binding protein 1 inhibits dentin formation in mice Open

Qian Xu, Jiahe Li, Hua Zhang, Shuzhen Wang, Chunlin Qin , et al. · 2024

Upon endoplasmic reticulum (ER) stress, inositol-requiring enzyme 1 (IRE1) is activated, which subsequently converts an unspliced X-box binding protein 1 ( XBP1U ) mRNA to a spliced mRNA that encodes a potent XBP1S transcription factor. XB…

Intracranial calcification in Fam20c-deficient mice recapitulates human Raine syndrome Open

Hua Zhang, Yongbo Lu, Phillip R. Kramer, M D Benson, Yi‐Shing Lisa Cheng , et al. · 2023

Long-acting PFI-2 small molecule release and multilayer scaffold design achieve extensive new formation of complex periodontal tissues with unprecedented fidelity Open

Huling Lyu, Xuefeng Zhou, Yunzhu Qian, Xiaohua Liu, Gokul Gopinathan , et al. · 2022

The faithful engineering of complex human tissues such as the bone/soft tissue/mineralized tissue interface in periodontal tissues requires innovative molecular cues in conjunction with tailored scaffolds. To address the loss of periodonta…

Enamel Defects Associated With Dentin Sialophosphoprotein Mutation in Mice Open

Tian Liang, Qian Xu, Hua Zhang, Shuzhen Wang, Thomas G.H. Diekwisch , et al. · 2021

Dentin sialophosphoprotein (DSPP) is an extracellular matrix protein that is highly expressed in odontoblasts, but only transiently expressed in presecretory ameloblasts during tooth development. We previously generated a knockin mouse mod…

Effect of high phosphate diet on the formation of dentin in Fam20c‐deficient mice Open

Hua Zhang, Qian Xu, Yongbo Lu, Chunlin Qin · 2021

FAM20C (family with sequence similarity 20‐member C), a kinase that phosphorylates secretory proteins, plays essential roles in various biological processes. In humans, mutations in FAM20C gene cause Raine syndrome, an autosomal recessive …

Constitutive expression of spliced XBP1 causes perinatal lethality in mice Open

Qian Xu, Hua Zhang, Shuzhen Wang, Chunlin Qin, Yongbo Lu · 2021

Summary Upon endoplasmic reticulum (ER) stress, inositol‐requiring enzyme 1 (IRE1) is activated and catalyzes nonconventional splicing of an unspliced X‐box binding protein 1 (XBP1U) mRNA to yield a spliced XBP1 (XBP1S) mRNA that encodes a…

Noggin inhibition of mouse dentinogenesis Open

Priyam Jani, Hua Zhang, M. Douglas Benson, Chunlin Qin · 2019

FAM20A is essential for amelogenesis, but is dispensable for dentinogenesis Open

Lili Li, Wuliji Saiyin, Hua Zhang, Shuzhen Wang, Qian Xu , et al. · 2019

Requirement of Hyaluronan Synthase-2 in Craniofacial and Palate Development Open

Yu Lan, Chunlin Qin, Rulang Jiang · 2019

Cleft palate is a common major birth defect resulting from disruption of palatal shelf growth, elevation, or fusion during fetal palatogenesis. Whereas the molecular mechanism controlling palatal shelf elevation is not well understood, a p…

Inactivation of FAM20B causes cell fate changes in annulus fibrosus of mouse intervertebral disc and disc defects via the alterations of TGF-β and MAPK signaling pathways Open

Wuliji Saiyin, Lili Li, Hua Zhang, Yongbo Lu, Chunlin Qin · 2019

Mutant Dentin Sialophosphoprotein Causes Dentinogenesis Imperfecta Open

Tian Liang, Hua Zhang, Qian Xu, S. Wang, Chunlin Qin , et al. · 2019

Dentin sialophosphoprotein (DSPP) is an extracellular matrix protein highly expressed by odontoblasts in teeth. DSPP mutations in humans may cause dentinogenesis imperfecta (DGI), an autosomal dominant dentin disorder. We recently generate…

Loss of Fam20c causes defects in the acinar and duct structure of salivary glands in mice Open

Nan Miao, Yuanbo Zhan, Yingying Xu, Haoze Yuan, Chunlin Qin , et al. · 2019

Family with sequence similarity 20‑member C (FAM20C), a recently characterized Golgi kinase, performs numerous biological functions by phosphorylating more than 100 secreted proteins. However, the role of FAM20C in the salivary glands rema…

High-Phosphate Diet Improved the Skeletal Development of Fam20c-Deficient Mice Open

Hua Zhang, Lili Li, Matthew J. Kesterke, Yongbo Lu, Chunlin Qin · 2019

FAM20C (family with sequence similarity 20 – member C) is a protein kinase that phosphorylates secretory proteins, including the proteins that are essential to the formation and mineralization of calcified tissues. Previously, we reported …

Inactivation of Fam20b in the neural crest‐derived mesenchyme of mouse causes multiple craniofacial defects Open

Xuenan Liu, Nan Li, Hua Zhang, Jing Liu, Nan Zhou , et al. · 2018

The glycosaminoglycan ( GAG ) chains attached to the core proteins of proteoglycans exert multiple roles, such as enriching signal molecules and regulating the binding of ligands to the corresponding receptors. A newly identified kinase – …

Transgenic expression of dentin phosphoprotein (DPP) partially rescued the dentin defects of DSPP-null mice Open

Hua Zhang, Xiaohua Xie, Peihong Liu, Tian Liang, Yongbo Lu , et al. · 2018

Mutations in the dentin sialophosphoprotein (DSPP) gene cause dentinogenesis imperfecta. After synthesis, DSPP is proteolytically processed into NH2- and COOH-terminal fragments. The NH2-terminal fragment of DSPP is highly glycosylated but…

The importance of a potential phosphorylation site in enamelin on enamel formation Open

Wenjuan Yan, Pan Ma, Ye Tian, Jingya Wang, Chunlin Qin , et al. · 2017

Enamelin (ENAM) has three putative phosphoserines (pSers) phosphorylated by a Golgi-associated secretory pathway kinase (FAM20C) based on their distinctive Ser-x-Glu (S-x-E) motifs. Fam20C-knockout mice show severe enamel defects similar t…

FAM20C regulates osteoblast behaviors and intracellular signaling pathways in a cell‐autonomous manner Open

Chao Liu, Hua Zhang, Priyam Jani, Xiaofang Wang, Yongbo Lu , et al. · 2017

Recent studies indicate that Family with sequence similarity 20 member C (FAM20C) catalyzes the phosphorylation of secreted proteins, and participates in a variety of biological processes, including cell proliferation, migration, mineraliz…

Probing the influence of SIBLING proteins on collagen-I fibrillogenesis and denaturation Open

Chengyu Jiang, Kevin M. Zurick, Chunlin Qin, Matthew T. Bernards · 2017

Bone tissue is comprised of collagen, non-collagenous proteins, and hydroxyapatite and the SIBLING (small integrin binding, N-linked glycoprotein) family of proteins is the primary group of non-collagenous proteins. By replicating the nati…

Specific ablation of mouse Fam20C in cells expressing type I collagen leads to skeletal defects and hypophosphatemia Open

Peihong Liu, Su Ma, Hua Zhang, Chao Liu, Yongbo Lu , et al. · 2017

FAM20C mutations in humans cause Raine syndrome and our previous studies showed that global inactivation of mouse Fam20C led to bone and dental defects. By crossbreeding 2.3 kb Col 1a1-Cre mice with Fam20C flox/flox mice, we created 2.3 kb…

Inactivation of Fam20B in Joint Cartilage Leads to Chondrosarcoma and Postnatal Ossification Defects Open

Pan Ma, Wenjuan Yan, Ye Tian, Jingya Wang, Jian Q. Feng , et al. · 2016

Loss of epithelial FAM20A in mice causes amelogenesis imperfecta, tooth eruption delay and gingival overgrowth Open

Lili Li, Peihong Liu, Xiaohua Xie, Su Ma, Chao Liu , et al. · 2016

FAM20A has been studied to a very limited extent. Mutations in human FAM20A cause amelogenesis imperfecta, gingival fibromatosis and kidney problems. It would be desirable to systemically analyse the expression of FAM20A in dental tissues …

Abrogation of epithelial BMP2 and BMP4 causes Amelogenesis Imperfecta by reducing MMP20 and KLK4 expression Open

Xiaohua Xie, Chao Liu, Hua Zhang, Priyam Jani, Yongbo Lu , et al. · 2016

Transgenic expression of dentin phosphoprotein inhibits skeletal development Open

Hua Zhang, P. Liu, S. Wang, C. Liu, Priyam Jani , et al. · 2016

Dentin sialophosphoprotein (DSPP) is proteolytically processed into an NH2-terminal fragment called dentin sialoprotein (DSP) and a COOH-terminal fragment known as dentin phosphoprotein (DPP). These two fragments are believed to perform di…

Repair of dentin defects from DSPP knockout mice by PILP mineralization Open

Hamid Nurrohman, Kunkio Saeki, Karina M. M. Carneiro, Yung‐Ching Chien, Sabra Djomehri , et al. · 2016

Accelerated enamel mineralization in Dspp mutant mice Open

Kostas Verdelis, Heather L. Szabo‐Rogers, Yang Xu, Rong Chong, Ryan Kang , et al. · 2016

Transgenic expression of Dspp partially rescued the long bone defects of Dmp1-null mice Open

Priyam Jani, Monica Prasad Gibson, Chao Liu, Hua Zhang, Xiaofang Wang , et al. · 2015

The LPV Motif Is Essential for the Efficient Export of Secretory DMP1 From the Endoplasmic Reticulum Open

Tian Liang, Tian Meng, Shuzhen Wang, Chunlin Qin, Yongbo Lu · 2015

Dentin matrix protein 1 (DMP1) is found abundantly in the extracellular matrices of bone and dentin. Secretory DMP1 begins with a tripeptide of leucine-proline-valine (LPV) after the endoplasmic reticulum (ER)-entry signal peptide is cleav…

Twist1 Is Essential for Tooth Morphogenesis and Odontoblast Differentiation Open

Tian Meng, Yanyu Huang, Shuzhen Wang, Hua Zhang, Paul C. Dechow , et al. · 2015

Twist1 is a basic helix-loop-helix-containing transcription factor that is expressed in the dental mesenchyme during the early stages of tooth development. To better delineate its roles in tooth development, we generated Twist1 conditional…

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