Journal of Orthopaedic Reports, 2022 Background: X-linked hereditary hypophosphatemic rickets (XHPR) is the dominant form of hereditary rickets characterized by alteration of phosphate metabolism and defective mineralization of the growth cartilage and the newly formed bone collagen.
Megremis Panos, Megremis Orestis
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Osteocytes and the pathogenesis of hypophosphatemic rickets
Frontiers in Endocrinology, 2022 Since phosphorus is a component of hydroxyapatite, its prolonged deprivation affects bone mineralization. Fibroblast growth factor 23 (FGF23) is essential for maintaining phosphate homeostasis and is mainly produced by osteocytes.
Miwa Yamazaki, Toshimi Michigami
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Enamel and dentin mineralization in familial hypophosphatemic rickets: a micro-CT study [PDF]
, 2015 Objectives: The aim of the present study was to analyse the mineralization pattern of enamel and dentin in patients affected by X-linked hypophosphatemic rickets (XLHR) using micro-CT (µCT), and to associate enamel and dentin mineralization in primary ...
Costa, F. W. G. +4 more
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The metabolic bone disease associated with the Hyp mutation is independent of osteoblastic HIF1α expression [PDF]
, 2017 Fibroblast growth factor-23 (FGF23) controls key responses to systemic phosphate increases through its phosphaturic actions on the kidney. In addition to stimulation by phosphate, FGF23 positively responds to iron deficiency anemia and hypoxia in rodent ...
Allen, Matt +5 more
core +9 more sources
Systemic Control of Bone Homeostasis by FGF23 Signaling [PDF]
, 2016 The regulation of phosphate metabolism as an influence on bone homeostasis is profound. Recent advances in understanding the systemic control of Fibroblast growth factor-23 (FGF23) has uncovered novel effectors of endocrine feedback loops for calcium ...
Clinkenbeard, Erica L. +1 more
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Indian Journal of Endocrinology and Metabolism, 2012 Hypophosphatemic rickets is a disorder of bone mineralization caused due to defects (inherited/acquired) in the renal handling of phosphorus. This group includes varied conditions, X-linked hypophosphatemic rickets being the most common inheritable form ...
Varsha S Jagtap +5 more
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Regulation of Phosphate Transporters and Novel Regulator of Phosphate Metabolism
Endocrines, 2023 Phosphorus is essential for all living organisms. It plays an important role in maintaining biological functions, such as energy metabolism, cell membrane formation, and bone mineralization.
Megumi Koike +4 more
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Conditional Deletion of Murine Fgf23: Interruption of the Normal Skeletal Responses to Phosphate Challenge and Rescue of Genetic Hypophosphatemia [PDF]
, 2016 The transgenic and knockout (KO) animals involving Fgf23 have been highly informative in defining novel aspects of mineral metabolism, but are limited by shortened lifespan, inability of spatial/temporal FGF23 control, and infertility of the global KO ...
Allen, Matthew R. +6 more
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Autoimmune hyperphosphatemic tumoral calcinosis in a patient with FGF23 autoantibodies [PDF]
, 2018 Hyperphosphatemic familial tumoral calcinosis (HFTC)/hyperostosis-hyperphosphatemia syndrome (HHS) is an autosomal recessive disorder of ectopic calcification due to deficiency of or resistance to intact fibroblast growth factor 23 (iFGF23). Inactivating
Burbelo +14 more
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SUN-345 Use of Anti-FGF23 Monoclonal Antibody in the Treatment of Children and Adolescents with X-Linked Hypophosphatemic Rickets [PDF]
J Endocr Soc, 2020 Cassinelli H +6 more
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