Parathyroid-specific deletion of Klotho unravels a novel calcineurin-dependent FGF23 signaling pathway that regulates PTH secretion

PLoS Genet. 2013;9(12):e1003975. doi: 10.1371/journal.pgen.1003975. Epub 2013 Dec 12.

Abstract

Klotho acts as a co-receptor for and dictates tissue specificity of circulating FGF23. FGF23 inhibits PTH secretion, and reduced Klotho abundance is considered a pathogenic factor in renal secondary hyperparathyroidism. To dissect the role of parathyroid gland resident Klotho in health and disease, we generated mice with a parathyroid-specific Klotho deletion (PTH-KL(-/-)). PTH-KL(-/-) mice had a normal gross phenotype and survival; normal serum PTH and calcium; unaltered expression of the PTH gene in parathyroid tissue; and preserved PTH response and sensitivity to acute changes in serum calcium. Their PTH response to intravenous FGF23 delivery or renal failure did not differ compared to their wild-type littermates despite disrupted FGF23-induced activation of the MAPK/ERK pathway. Importantly, calcineurin-NFAT signaling, defined by increased MCIP1 level and nuclear localization of NFATC2, was constitutively activated in PTH-KL(-/-) mice. Treatment with the calcineurin-inhibitor cyclosporine A abolished FGF23-mediated PTH suppression in PTH-KL(-/-) mice whereas wild-type mice remained responsive. Similar results were observed in thyro-parathyroid explants ex vivo. Collectively, we present genetic and functional evidence for a novel, Klotho-independent, calcineurin-mediated FGF23 signaling pathway in parathyroid glands that mediates suppression of PTH. The presence of Klotho-independent FGF23 effects in a Klotho-expressing target organ represents a paradigm shift in the conceptualization of FGF23 endocrine action.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Calcineurin / metabolism
  • Calcineurin Inhibitors
  • Calcium / blood
  • Cyclosporine / pharmacology
  • Fibroblast Growth Factor-23
  • Fibroblast Growth Factors / genetics*
  • Fibroblast Growth Factors / metabolism
  • Gene Expression Regulation / drug effects
  • Humans
  • Kidney / metabolism
  • Klotho Proteins
  • Membrane Proteins / genetics*
  • Membrane Proteins / metabolism
  • Mice
  • Parathyroid Glands / metabolism
  • Parathyroid Hormone / blood
  • Parathyroid Hormone / genetics*
  • Sequence Deletion
  • Signal Transduction / genetics*
  • Vitamin D / metabolism

Substances

  • Calcineurin Inhibitors
  • FGF23 protein, human
  • Fgf23 protein, mouse
  • Klb protein, mouse
  • Membrane Proteins
  • Parathyroid Hormone
  • Vitamin D
  • Fibroblast Growth Factors
  • Fibroblast Growth Factor-23
  • Cyclosporine
  • Calcineurin
  • Klotho Proteins
  • Calcium