Fibroblast Growth Factor-23 and Matrix Extracellular Phosphoglycoprotein Levels in Healthy Children and, Pregnant and Puerperal Women

2019 ◽  
Vol 92 (5) ◽  
pp. 302-310
Author(s):  
Ahu Ozsen ◽  
Andrzej Furman ◽  
Tulay Guran ◽  
Abdullah Bereket ◽  
Serap Turan
Keyword(s):  
Growth Factor ◽  
Fibroblast Growth Factor ◽  
Fibroblast Growth Factor 23 ◽  
Healthy Children ◽  
Fibroblast Growth ◽  
Matrix Extracellular Phosphoglycoprotein

scholarly journals The Role of Mutant UDP-N-Acetyl-α-d-Galactosamine-Polypeptide N-Acetylgalactosaminyltransferase 3 in Regulating Serum Intact Fibroblast Growth Factor 23 and Matrix Extracellular Phosphoglycoprotein in Heritable Tumoral Calcinosis

2006 ◽  
Vol 91 (10) ◽  
pp. 4037-4042 ◽  
Author(s):  
Holly J. Garringer ◽  
Corinne Fisher ◽  
Tobias E. Larsson ◽  
Siobhan I. Davis ◽  
Daniel L. Koller ◽  
...  
Keyword(s):  
Growth Factor ◽  
Combination Therapy ◽  
Fibroblast Growth Factor ◽  
Fibroblast Growth Factor 23 ◽  
High Serum ◽  
Biologically Active ◽  
Tumoral Calcinosis ◽  
Fibroblast Growth ◽  
Loss Of Function ◽  
Matrix Extracellular Phosphoglycoprotein

Abstract Context: Familial tumoral calcinosis (TC) results from disruptions in phosphate metabolism and is characterized by high serum phosphate with normal or elevated 1,25 dihydroxyvitamin vitamin D concentrations and ectopic and vascular calcifications. Recessive loss-of-function mutations in UDP-N-acetyl-α-d-galactosamine-polypeptide N-acetylgalactosaminyltransferase 3 (GALNT3) and fibroblast growth factor-23 (FGF23) result in TC. Objective: The objective of the study was to determine the relationship between GALNT3 and FGF23 in familial TC. Design, Setting, and Patients: We assessed the major biochemical defects and potential genes involved in patients with TC. Intervention: Combination therapy consisted of the phosphate binder Sevelamer and the carbonic anhydrase inhibitor acetazolamide. Results: We report a patient homozygous for a GALNT3 exon 1 deletion, which is predicted to truncate the encoded protein. This patient had high serum FGF23 concentrations when assessed with a C-terminal FGF23 ELISA but low-normal FGF23 levels when tested with an ELISA for intact FGF23 concentrations. Matrix extracellular phosphoglycoprotein has been identified as a possible regulator of phosphate homeostasis. Serum matrix extracellular phosphoglycoprotein levels, however, were normal in the family with GALNT3-TC and a kindred with TC carrying the FGF23 S71G mutation. The tumoral masses of the patient with GALNT3-TC completely resolved after combination therapy. Conclusions: Our findings demonstrate that GALNT3 inactivation in patients with TC leads to inadequate production of biologically active FGF23 as the most likely cause of the hyperphosphatemic phenotype. Furthermore, combination therapy may be effective for reducing the tumoral burden associated with familial TC.

scholarly journals Human recombinant endopeptidase PHEX has a strict S1' specificity for acidic residues and cleaves peptides derived from fibroblast growth factor-23 and matrix extracellular phosphoglycoprotein

2003 ◽  
Vol 373 (1) ◽  
pp. 271-279 ◽  
Author(s):  
Marcelo CAMPOS ◽  
Constance COUTURE ◽  
Izaura Y. HIRATA ◽  
Maria A. JULIANO ◽  
Thomas P. LOISEL ◽  
...  
Keyword(s):  
Growth Factor ◽  
Substrate Specificity ◽  
Fibroblast Growth Factor ◽  
Fibroblast Growth Factor 23 ◽  
Fibroblast Growth ◽  
Amino Acid Residues ◽  
Acceptor Pair ◽  
Phex Gene ◽  
Strict Requirement ◽  
Matrix Extracellular Phosphoglycoprotein

The PHEX gene (phosphate-regulating gene with homologies to endopeptidases on the X chromosome) encodes a protein (PHEX) with structural homologies to members of the M13 family of zinc metallo-endopeptidases. Mutations in the PHEX gene are responsible for X-linked hypophosphataemia in humans. However, the mechanism by which loss of PHEX function results in the disease phenotype, and the endogenous PHEX substrate(s) remain unknown. In order to study PHEX substrate specificity, combinatorial fluorescent-quenched peptide libraries containing o-aminobenzoic acid (Abz) and 2,4-dinitrophenyl (Dnp) as the donor–acceptor pair were synthesized and tested as PHEX substrates. PHEX showed a strict requirement for acidic amino acid residues (aspartate or glutamate) in S1´ subsite, with a strong preference for aspartate. Subsites S2´, S1 and S2 exhibited less defined specificity requirements, but the presence of leucine, proline or glycine in P2´, or valine, isoleucine or histidine in P1 precluded hydrolysis of the substrate by the enzyme. The peptide Abz-GFSDYK(Dnp)-OH, which contains the most favourable residues in the P2 to P2´ positions, was hydrolysed by PHEX at the N-terminus of aspartate with a kcat/Km of 167 mM−1·s−1. In addition, using quenched fluorescence peptides derived from fibroblast growth factor-23 and matrix extracellular phosphoglycoprotein sequences flanked by Abz and N-(2,4-dinitrophenyl)ethylenediamine, we showed that these physiologically relevant proteins are potential PHEX substrates. Finally, our results clearly indicate that PHEX does not have neprilysin-like substrate specificity.

Fibroblast growth factor 23 and Klotho serum levels in healthy children

Bone ◽  
2014 ◽  
Vol 66 ◽  
pp. 8-14 ◽  
Author(s):  
Despoina Gkentzi ◽  
Alexandra Efthymiadou ◽  
Dimitra Kritikou ◽  
Dionisios Chrysis
Keyword(s):  
Growth Factor ◽  
Fibroblast Growth Factor ◽  
Fibroblast Growth Factor 23 ◽  
Serum Levels ◽  
Healthy Children ◽  
Fibroblast Growth

scholarly journals Fibroblast growth factor 23 (FGF-23) and chronic kidney disease in children

2021 ◽  
Vol 11-12 (221-222) ◽  
pp. 43-48
Author(s):  
Altynay Balmukhanova ◽  
◽  
Kairat Kabulbayev ◽  
Assiya Kanatbayeva ◽  
◽  
...  
Keyword(s):  
Chronic Kidney Disease ◽  
Growth Factor ◽  
Kidney Disease ◽  
Fibroblast Growth Factor ◽  
Fibroblast Growth Factor 23 ◽  
Healthy Children ◽  
Fibroblast Growth ◽  
Mineral Bone Disorder ◽  
Fgf 23 ◽  
Stage 1

Chronic kidney disease (CKD) in children is a complex medical and social problem in healthcare. One of the serious complications is mineral bone disorder (MBD), the pathogenesis of which is related to a new biomarker of bone origin - fibroblast growth factor 23 (FGF-23). The aim. To study the features of fibroblast growth factor 23 (FGF-23) in children with chronic kidney disease. Material and methods. A cross-sectional study was carried out on 73 children with CKD and 14 healthy children. Inclusion criteria: chronic kidney disease stage 1-5, written informed consent of the participants. The exclusion criteria: tubulopathy, infectious and inflammatory processes, oncological diseases, kidney transplant, condition after surgery, taking glucocorticosteroids, calcium and vitamin D drugs. We took fasting blood samples of participants and carried out an enzyme-linked immunosorbent assay in order determine the level of FGF-23 (Biomedica Medizinprodukte GmbH, Austria). The obtained data were analyzed using IBM SPSS, version 22 (New York, USA). Results and discussion. In healthy children, the median (Q1-Q3) level of FGF-23 in serum was 0.65 (0.22-0.98) pmol/l, in patients with stage 1 CKD it was 0.65 (0.22-1.08) pmol/l. At stage 2, the level of FGF-23 significantly increased in comparison with healthy individuals and with patients of stage 1, p≤0.05. Further, there is a gradual increase by stages, p≤0.05. Thus, in stage 3 patients, the median FGF-23 value was 1.9 (1.15-3.5) pmol/l, at stage 4 - 3.55 (2.48-6.35) pmol/l, at 5 stages - 14 (7.5-18.75) pmol/l. As a percentage, there were 7.1% of patients at the stage 1 with increased levels of phosphatonin, at stage 2 - 53.3%, at stage 3 - 69.2%, respectively. At stages 4 and 5, absolutely 100% of patients had high levels of FGF-23. At the same time, FGF-23 did not depend on gender, age, birth weight and type of renal replacement therapy at stage 5, p>0.05. Conclusions. Thus, in our study, we determined the features of changes FGF-23 in serum in children at various stages of CKD. The obtained results allow us to consider FGF-23 as a predictor of the clinical course of CKD. Keywords: fibroblast growth factor 23, phosphatonin, pediatric nephrology, chronic kidney disease, mineral-bone disorder.

Sex and iron modify fibroblast growth factor 23 concentrations in 1-year-old children

2017 ◽  
Author(s):  
Elisa Holmlund-Suila ◽  
Maria Enlund-Cerullo ◽  
Saara Valkama ◽  
Helena Hauta-alus ◽  
Jenni Rosendahl ◽  
...  
Keyword(s):  
Growth Factor ◽  
Fibroblast Growth Factor ◽  
Fibroblast Growth Factor 23 ◽  
Fibroblast Growth
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