Article
Biochemistry & Molecular Biology
Ke Wang, Yinshi Ren, Shuxian Lin, Yan Jing, Chi Ma, Jun Wang, X. Baozhi Yuan, Xianglong Han, Hu Zhao, Zheng Wang, Minghao Zheng, Yin Xiao, Lin Chen, Bjorn Reino Olsen, Jian Q. Feng
Summary: This study found that osteocytes, not osteoblasts, directly build mineralized bone structures, and that osteoblasts in Dmp1-null or Hyp mice produce aberrant and poorly mineralized bone. Additionally, the constitutive expression of beta-catenin in osteocytes can recapitulate a similar osteomalacia phenotype as seen in Dmp1 null or Hyp mice. Thus, the conclusion is that osteocytes directly build bone, challenging the existing dogma.
INTERNATIONAL JOURNAL OF BIOLOGICAL SCIENCES
(2021)
Article
Engineering, Biomedical
F. Machla, V Sokolova, V Platania, O. Prymak, K. Kostka, B. Kruse, M. Agrymakis, S. Pasadaki, A. Kritis, K. Alpantaki, M. Vidaki, M. Chatzinikolaidou, M. Epple, A. Bakopoulou
Summary: Tissue engineering provides new treatment approaches for regenerating lost dental tissues, and this study investigated the characteristics of EDTA-treated, freeze-dried dentin matrices (HTFD scaffolds) conditioned with calcium phosphate nanoparticles (NPs) bearing dentinogenesis-inducing factors. The synthesized NPs showed efficient transfection of dental pulp stem cells (DPSCs), and HTFD/NPs constructs induced odontogenic shift of DPSCs, preserving cell viability and leading to the reconstruction of the ortho-dentin/odontoblastic layer barrier.
ACTA BIOMATERIALIA
(2023)
Article
Dentistry, Oral Surgery & Medicine
Hua Zhang, Qian Xu, Yongbo Lu, Chunlin Qin
Summary: This study demonstrates that a high phosphate diet improves dentin volume fraction and mineral density in Fam20c-deficient mice, preventing pulp infection and dental abscesses. These results suggest that hypophosphatemia is a significant contributor to dentin defects in Fam20c-deficient subjects.
EUROPEAN JOURNAL OF ORAL SCIENCES
(2021)
Editorial Material
Hematology
Laura M. Calvi
Summary: This study highlights the novel role of FGF-23 in G-CSF-stimulated mobilization of HSPCs, shedding light on the connection between phosphate metabolism and hematopoietic stem cells.
Review
Endocrinology & Metabolism
Pablo Florenzano, Iris R. Hartley, Macarena Jimenez, Kelly Roszko, Rachel I. Gafni, Michael T. Collins
Summary: Tumor-induced osteomalacia is a rare paraneoplastic syndrome caused by tumoral production of FGF23, characterized by hypophosphatemia, abnormal vitamin D levels, and elevated FGF23. Treatment includes surgical resection, medical therapy, and novel approaches, but locating the tumor can be challenging.
CALCIFIED TISSUE INTERNATIONAL
(2021)
Article
Multidisciplinary Sciences
Ashwini Kumar Nepal, Hubertus W. van Essen, Christianne M. A. Reijnders, Paul Lips, Nathalie Bravenboer
Summary: Mechanical loading of bone affects factors of phosphate homeostasis by modulating the gene expression of Fgf23, Mepe, Dmp1, Phex, Cyp27b1, and Vdr. It results in a decrease in Fgf23 gene expression and serum FGF23 levels, as well as an increase in Dmp1 and Mepe gene expression. However, it does not affect the gene expression of Phex, Cyp27b1, and Vdr.
Article
Medical Laboratory Technology
Marie-Eva Pickering, Damien Bouvier, Antoine Puravet, Martin Soubrier, Vincent Sapin, Charlotte Oris
Summary: This case report describes a rare case of hypophosphatemic osteomalacia associated with pancreatic cancer, highlighting the importance of plasma FGF23 measurement in patients with hypophosphatemia, renal phosphate wasting, and low calcitriol level.
CLINICAL BIOCHEMISTRY
(2022)
Review
Genetics & Heredity
Claudia Maria Jurca, Oana Iuhas, Kinga Kozma, Codruta Diana Petchesi, Dana Carmen Zaha, Marius Bembea, Sanziana Jurca, Corina Paul, Alexandru Daniel Jurca
Summary: X-linked hypophosphatemia is a genetic disorder characterized by a gene mutation that inactivates the degradation of FGF23, leading to decreased tubular reabsorption of phosphorus. Symptoms include growth delay and bone pain. Treatment with Burosumab has shown positive results in improving the condition of patients.
Article
Endocrinology & Metabolism
Rafi Brener, Leonid Zeitlin, Yael Lebenthal, Avivit Brener
Summary: This prospective case-control study explored the effect of burosumab, an anti-FGF23 antibody, on dental health of children with X-linked hypophosphatemia (XLH). One year of treatment led to improvement of height and healing of rickets in XLH patients, and those achievements were maintained after three years of treatment. Dental morphology of XLH patients showed increased pulp coronal ratios compared to controls, and did not attain the decrease expected with age after three years of treatment. Some patients experienced recurrent dental abscesses during the treatment.
FRONTIERS IN ENDOCRINOLOGY
(2022)
Article
Endocrinology & Metabolism
Anne Gladding, Vivian Szymczuk, Bethany A. Auble, Alison M. Boyce
Summary: This study reports the first case of a 7-year-old boy with FD/MAS treated with burosumab. The patient achieved normalization of serum phosphorus, marked improvement in alkaline phosphatase levels, and showed encouraging clinical response without any observed adverse effects. Future studies are needed to further explore the safety and efficacy of burosumab in the pediatric population with FD/MAS.
Article
Endocrinology & Metabolism
Benedikt Schaefer, Heinz Zoller, Myles Wolf
Summary: This study found that treatment with FCM was the only consistent risk factor for hypophosphatemia. Patients who developed severe or persistent hypophosphatemia after FCM treatment had more severe derangements in bone and mineral metabolism. Changes in bone biomarkers continued beyond resolution of hypophosphatemia, indicating ongoing effects on bone that may explain the association of FCM with osteomalacia and fractures.
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
(2022)
Article
Endocrinology & Metabolism
Thomas J. Weber, Erik A. Imel, Thomas O. Carpenter, Munro Peacock, Anthony A. Portale, Joel Hetzer, J. Lawrence Merritt, Karl Insogna
Summary: The study aimed to evaluate the safety, immunogenicity, and clinical response of long-term burosumab treatment in adult XLH patients. Results showed improvements in fasting serum phosphate levels, bone turnover markers, and patient-reported outcomes, supporting burosumab as a safe and effective long-term treatment option for XLH patients.
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
(2022)
Review
Endocrinology & Metabolism
Luisella Cianferotti, Chiara Delli Poggi, Francesco Bertoldo, Carla Caffarelli, Chiara Crotti, Davide Gatti, Sandro Giannini, Stefano Gonnelli, Maurizio Mazzantini, Viapiana Ombretta, Stefania Sella, Angela Setti, Massimo Varenna, Francesca Zucchi, Maria Luisa Brandi
Summary: Tumor-induced osteomalacia is a rare mineral metabolism disorder characterized by hypophosphatemia. This study found that a significant number of TIO patients remain undiagnosed or persist after surgery, making them potential candidates for burosumab treatment.
Article
Cell Biology
Kohei Naniwa, Katsutoshi Hirose, Yu Usami, Kenji Hata, Rikita Araki, Narikazu Uzawa, Toshihisa Komori, Satoru Toyosawa
Summary: FAM20C phosphorylates secretory proteins and plays essential roles in bone and tooth formation. The direct role of FAM20C remains unknown due to the masking effect of hypophosphatemia in Fam20C-deficient mice. In this study, we demonstrated that odontoblastic FAM20C-mediated phosphorylation regulates dentin formation and odontoblast differentiation using Fam20C-Tg mice.
JOURNAL OF MOLECULAR HISTOLOGY
(2023)
Review
Genetics & Heredity
Francis H. Glorieux, Lynda F. Bonewald, Nicholas C. Harvey, Marjolein C. H. van der Meulen
Summary: Recent progress has been made in understanding the mechanisms of bone growth and development, particularly in the crosstalk between muscle and bone. However, limited knowledge exists on how these mechanisms apply or are compromised in pathological states. This review summarizes the research opinions and potential outcomes for XLH patients.
ORPHANET JOURNAL OF RARE DISEASES
(2022)
Article
Dentistry, Oral Surgery & Medicine
X. Xie, J. Wang, K. Wang, C. Li, S. Zhang, D. Jing, C. Xu, X. Wang, H. Zhao, J. Q. Feng
JOURNAL OF DENTAL RESEARCH
(2019)
Article
Cell Biology
Lora A. Shiflett, Leann M. Tiede-Lewis, Yixia Xie, Yongbo Lu, Eleanor C. Ray, Sarah L. Dallas
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2019)
Article
Cell Biology
Lili Li, Wuliji Saiyin, Hua Zhang, Suzhen Wang, Qian Xu, Chunlin Qin, Yongbo Lu
JOURNAL OF MOLECULAR HISTOLOGY
(2019)
Article
Biochemistry & Molecular Biology
Wuliji Saiyin, Lili Li, Hua Zhang, Yongbo Lu, Chunlin Qin
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
(2019)
Article
Biology
Jingyi Wu, Ye Tian, Lu Han, Chao Liu, Tianyu Sun, Ling Li, Yanlei Yu, Bikash Lamichhane, Rena N. D'Souza, Sarah E. Millar, Robb Krumlauf, David M. Ornitz, Jian Q. Feng, Ophir Klein, Hu Zhao, Fuming Zhang, Robert J. Linhardt, Xiaofang Wang
Article
Developmental Biology
Jingyi Wu, Hong Li, Lu Han, Tianyu Sun, Ye Tian, Xiaofang Wang
GENE EXPRESSION PATTERNS
(2020)
Article
Dentistry, Oral Surgery & Medicine
Hua Zhang, Qian Xu, Yongbo Lu, Chunlin Qin
Summary: This study demonstrates that a high phosphate diet improves dentin volume fraction and mineral density in Fam20c-deficient mice, preventing pulp infection and dental abscesses. These results suggest that hypophosphatemia is a significant contributor to dentin defects in Fam20c-deficient subjects.
EUROPEAN JOURNAL OF ORAL SCIENCES
(2021)
Article
Developmental Biology
Qian Xu, Hua Zhang, Suzhen Wang, Chunlin Qin, Yongbo Lu
Summary: XBP1S is a crucial mediator in the IRE1 branch for alleviating ER stress. Precise control of XBP1S production is essential for normal mouse development as demonstrated in experiments.
Article
Anatomy & Morphology
Kaoru Fujikawa, Naoko Nonaka, Xiaofang Wang, Shunichi Shibata
Summary: The expression patterns of syndecan-1, 2, 3, and 4 mRNAs during late tooth germ formation were investigated. Syndecan-1 mRNA was mainly expressed in specific tooth germ regions and its expression in dental epithelium increased during the postnatal periods. Syndecan-3 mRNA was found to be involved in dentinogenesis, while syndecan-2 showed enhanced expression with hard tissue formation. Syndecan-4 exhibited similar expression patterns to syndecan-3.
ANATOMICAL SCIENCE INTERNATIONAL
(2022)
Review
Cell Biology
Jiawen Chen, Tianyu Sun, Yan You, Buling Wu, Xiaofang Wang, Jingyi Wu
Summary: Proteoglycans are essential for regulating stem cell homeostasis and show potential for being utilized as novel glycomics carriers or bio-active molecules in bone regeneration. Recent research has outlined their distinct profiles and functions in maintaining the balance between self-renewal and differentiation of stem cells.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Cell Biology
Xin Fang, Nan Ni, Xiaofang Wang, Yanan Tian, Ivan Ivanov, Monique Rijnkels, Kayla J. Bayless, John P. Lydon, Qinglei Li
Summary: EZH2 has dual roles in the development of endometrial cancer, functioning as an oncogene in the early stages but also leading to unfavorable outcomes during disease progression, characterized by exacerbated epithelial stratification and heightened inflammatory response.
Article
Biochemistry & Molecular Biology
Changchun Dong, Bikash Lamichhane, Hajime Yamazaki, Brent Vasquez, Jingya Wang, Yongxu Zhang, Jian Q. Feng, Henry C. Margolis, Elia Beniash, Xiaofang Wang
Summary: This study generated two lines of Enam knock-in mice using the CRISPR-Cas9 method to eliminate or mimic the phosphorylation state of Ser(55) in ENAM. The results showed that Ser(55) phosphorylation is essential for ENAM function during enamel formation.
Article
Biology
Bhaba K. Das, Lei Wang, Toshifumi Fujiwara, Jian Zhou, Nukhet Aykin-Burns, Kimberly J. Krager, Renny Lan, Samuel G. Mackintosh, Ricky Edmondson, Michael L. Jennings, Xiaofang Wang, Jian Q. Feng, Tomasa Barrientos, Jyoti Gogoi, Aarthi Kannan, Ling Gao, Weirong Xing, Subburaman Mohan, Haibo Zhao
Summary: Increased intracellular iron through Tfr1-mediated iron uptake is essential for mitochondrial metabolism and cytoskeletal organization in osteoclasts, leading to proper bone resorption and skeletal homeostasis.
Article
Multidisciplinary Sciences
Changchun Dong, Bikash Lamichhane, Yongxu Zhang, Xiaofang Wang
Summary: This study describes a novel method for visualizing and analyzing label-retaining cells (LRCs) in mouse incisors. By using tissue clearing and whole-mount EdU staining, combined with confocal microscopy and imaging software for 3D reconstruction, this method enables unbiased quantitation compared to traditional LRCs analysis on sectioned slides.
JOVE-JOURNAL OF VISUALIZED EXPERIMENTS
(2022)
Article
Biochemistry & Molecular Biology
Zheng Wang, Chi Ma, Diane Chen, Caitlin Haslett, Chunmei Xu, Changchun Dong, Xiaofang Wang, Minghao Zheng, Yan Jing, Jian Q. Feng
Summary: This study reveals that tendon cells directly form fibrocartilage through cell transdifferentiation, shedding light on the mechanisms underlying enthesopathy and sporting injuries.
INTERNATIONAL JOURNAL OF BIOLOGICAL SCIENCES
(2023)