The DOI matches its Crossref record; 43 of the 43 references it cites were found in a registry or an index.
Dynamic regulation of FGF23 by Fam20C phosphorylation, GalNAc-T3 glycosylation, and furin proteolysis
Open accessAbstract
Significance: The family with sequence similarity 20, member C (Fam20C) is a secretory pathway-specific kinase that phosphorylates secreted proteins on Ser-x-Glu/pSer motifs. Mutations in human FAM20C cause a devastating childhood disorder known as Raine syndrome. Some patients with FAM20C mutations as well as Fam20C KO mice develop hypophosphatemia due to elevated levels of the phosphate-regulating hormone FGF23. In this paper, we show that Fam20C phosphorylates FGF23 on a Ser-x-Glu motif that lies within a critical region of the hormone. The phosphorylation promotes FGF23 proteolysis by furin by blocking O-glycosylation by polypeptide N -acetylgalactosaminyltransferase 3. Our results have important implications for patients with abnormalities in phosphate homeostasis.
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A Programmable Dual-RNA–Guided DNA Endonuclease in Adaptive Bacterial Immunity
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Multiplex Genome Engineering Using CRISPR/Cas Systems
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Autosomal dominant hypophosphataemic rickets is associated with mutations in FGF23
White, K. E., Evans, W. E., O'Riordan, J. L. H., Speer, M. C., Econs, M. J., Lorenz-Depiereux, B., Grabowski, M., Meitinger, T., & Strom, T. M. (2000) · Nature Genetics · doi:10.1038/81664 · Cited by 1,528
Self-citationRegulation of Fibroblast Growth Factor-23 Signaling by Klotho
Kurosu, H., Ogawa, Y., Miyoshi, M., Yamamoto, M., Nandi, A., Rosenblatt, K. P., Baum, M. G., Schiavi, S., Hu, M.-C., Moe, O. W., et al. (2006) · Journal of Biological Chemistry · doi:10.1074/jbc.c500457200 · Cited by 1,366
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“DMP1 appears to regulate FGF23 expression indirectly, as loss of Dmp1 in vivo impairs the maturation of osteoblasts to osteocytes through unknown mechanisms and results in highly elevated FGF23 mRNA as well as circulating protein (22).”
BackgroundSelf-citationThe origins of protein phosphorylation
Cohen, P. (2002) · Nature Cell Biology · doi:10.1038/ncb0502-e127 · Cited by 1,139
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Phosphorylated Proteins and Control over Apatite Nucleation, Crystal Growth, and Inhibition
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Regulation of Phosphate Homeostasis by PTH, Vitamin D, and FGF23
Bergwitz, C., & Jüppner, H. (2010) · Annual Review of Medicine · doi:10.1146/annurev.med.051308.111339 · Cited by 741
Cited by
Global view of human protein glycosylation pathways and functions
Schjoldager, K. T., Narimatsu, Y., Joshi, H. J., & Clausen, H. (2020) · Nature Reviews Molecular Cell Biology · doi:10.1038/s41580-020-00294-x · Cited by 1,309
FGF23 signalling and physiology
Ho, B. B., & Bergwitz, C. (2021) · Journal of Molecular Endocrinology · doi:10.1530/jme-20-0178 · Cited by 180
Impact of Phosphorus-Based Food Additives on Bone and Mineral Metabolism
Gutiérrez, O. M., Luzuriaga-McPherson, A., Lin, Y., Gilbert, L. C., Ha, S.-W., & Beck, G. R. (2015) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2015-2279 · Cited by 82
Harvey, D. J. (2018) · Mass Spectrometry Reviews · doi:10.1002/mas.21530 · Cited by 61
Phosphate-sensing and regulatory mechanism of FGF23 production
Takashi, Y., & Fukumoto, S. (2020) · Journal of Endocrinological Investigation · doi:10.1007/s40618-020-01205-9 · Cited by 42
Metadata
- Authors
- Tagliabracci, V. S., Engel, J. L., Wiley, S. E., Xiao, J., Gonzalez, D. J., Nidumanda Appaiah, H., Koller, A., Nizet, V., White, K. E., & Dixon, J. E.
- Venue
- Proceedings of the National Academy of Sciences
- Published
- 2014-03-26
- Volume
- 111
- Issue
- 15
- Pages
- 5520-5525
- ISSN
- 0027-8424 (ISSN-L)
- 1091-6490
- OpenAlex
- W1983579422
- Cited by
- 327
- References
- 43
- FWCI
- 19.65
- Type
- journal article
- Language
- en
- Open access
- bronze
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- CoChat. (2026). Citation check: Dynamic regulation of FGF23 by Fam20C phosphorylation, GalNAc-T3 glycosylation, and furin proteolysis. https://app.cochat.ai/papers/10.1073/pnas.1402218111
- The paper
- Tagliabracci, V. S., Engel, J. L., Wiley, S. E., Xiao, J., Gonzalez, D. J., Nidumanda Appaiah, H., Koller, A., Nizet, V., White, K. E., & Dixon, J. E. (2014). Dynamic regulation of FGF23 by Fam20C phosphorylation, GalNAc-T3 glycosylation, and furin proteolysis. Proceedings of the National Academy of Sciences. https://doi.org/10.1073/pnas.1402218111
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