Inducible podocyte-specific deletion of CTCF drives progressive kidney disease and bone abnormalities

JCI Insight. 2018 Feb 22;3(4):e95091. doi: 10.1172/jci.insight.95091.

Abstract

Progressive chronic kidney diseases (CKDs) are on the rise worldwide. However, the sequence of events resulting in CKD progression remain poorly understood. Animal models of CKD exploring these issues are confounded by systemic toxicities or surgical interventions to acutely induce kidney injury. Here we report the generation of a CKD mouse model through the inducible podocyte-specific ablation of an essential endogenous molecule, the chromatin structure regulator CCCTC-binding factor (CTCF), which leads to rapid podocyte loss (iCTCFpod-/-). As a consequence, iCTCFpod-/- mice develop severe progressive albuminuria, hyperlipidemia, hypoalbuminemia, and impairment of renal function, and die within 8-10 weeks. CKD progression in iCTCFpod-/- mice leads to high serum phosphate and elevations in fibroblast growth factor 23 (FGF23) and parathyroid hormone that rapidly cause bone mineralization defects, increased bone resorption, and bone loss. Dissection of the timeline leading to glomerular pathology in this CKD model led to the surprising observation that podocyte ablation and the resulting glomerular filter destruction is sufficient to drive progressive CKD and osteodystrophy in the absence of interstitial fibrosis. This work introduces an animal model with significant advantages for the study of CKD progression, and it highlights the need for podocyte-protective strategies for future kidney therapeutics.

Keywords: Chronic kidney disease; Endocrinology; Nephrology.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Bone Resorption / blood
  • Bone Resorption / etiology*
  • Bone Resorption / pathology
  • CCCTC-Binding Factor / deficiency*
  • CCCTC-Binding Factor / genetics
  • Calcification, Physiologic / genetics
  • Disease Models, Animal*
  • Disease Progression
  • Female
  • Fibroblast Growth Factor-23
  • Fibroblast Growth Factors / blood
  • Glomerular Filtration Rate
  • Humans
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Parathyroid Hormone / blood
  • Podocytes / pathology*
  • Renal Insufficiency, Chronic / blood
  • Renal Insufficiency, Chronic / complications
  • Renal Insufficiency, Chronic / genetics
  • Renal Insufficiency, Chronic / pathology*

Substances

  • CCCTC-Binding Factor
  • Ctcf protein, mouse
  • FGF23 protein, human
  • Fgf23 protein, mouse
  • Parathyroid Hormone
  • Fibroblast Growth Factors
  • Fibroblast Growth Factor-23