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Cited by 327

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 access
Tagliabracci, Vincent S., Engel, James L., Wiley, Sandra E., Xiao, Junyu, Gonzalez, David J., Nidumanda Appaiah, Hitesh, Koller, Antonius, Nizet, Victor, White, Kenneth E., Dixon, Jack E.·2014·Proceedings of the National Academy of Sciences

Abstract

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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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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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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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

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