Effect of celastrol on the progression of polycystic kidney disease in a Pkd1-deficient mouse model

Life Sci. 2018 Nov 1:212:70-79. doi: 10.1016/j.lfs.2018.09.047. Epub 2018 Sep 27.

Abstract

Aims: Celastrol, a naturally occurring pentacyclic triterpene, has attracted considerable interest because it exhibits potent anti-inflammatory and anti-tumor properties. However, the effects of celastrol in autosomal dominant polycystic kidney disease (ADPKD) remain uninvestigated.

Main methods: We determined the effects of celastrol on ADPKD progression in a novel Pkd1-hypomorphic mouse model by intraperitoneal injection (postnatal day 35-63).

Key findings: Pkd1 miRNA transgenic (Pkd1 miR TG) mice treated with 1 mg/kg/day of celastrol exhibited a lower renal cystic index (by 21.5%) than the vehicle-treated controls, but the fractional kidney weights and blood urea nitrogen levels were not significantly affected with celastrol treatment. At a high dose (2 mg/kg/day), celastrol caused marginal weight loss in the treated mice and had no significant effect on renal cystogenesis, thus indicating a potential toxic effect. We further identified that celastrol increased the phosphorylation level of adenosine monophosphate-activated protein kinase (AMPK) in the cystic kidneys. Moreover, celastrol reduced the renal mRNA expression levels of tumor necrosis factor-α, interleukin-1β, P2RX7, F4/80, CD68, transforming growth factor-β, collagen-1, and fibronectin, which were high in the Pkd1 miR TG mice. Immunohistological analysis revealed that celastrol suppressed macrophage infiltration in the cystic kidneys; however, the renal fibrosis scores and proliferation indices remained high.

Significance: These results indicate that celastrol could be a potent anti-inflammatory agent and a natural AMPK enhancer. However, celastrol has only modest effects on renal cystogenesis and has a narrow therapeutic window. Further studies are needed to clarify whether celastrol has the potential for the treatment of ADPKD.

Keywords: Celastrol; Inflammation; Macrophage; Polycystic kidney disease.

MeSH terms

  • Animals
  • Cell Proliferation / drug effects
  • Cysts / drug therapy*
  • Cysts / metabolism
  • Cysts / pathology
  • Disease Progression
  • Female
  • Inflammation Mediators / metabolism*
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Transgenic
  • Pentacyclic Triterpenes
  • Polycystic Kidney, Autosomal Dominant / drug therapy*
  • Polycystic Kidney, Autosomal Dominant / metabolism
  • Polycystic Kidney, Autosomal Dominant / pathology
  • TRPP Cation Channels / physiology*
  • Triterpenes / pharmacology*

Substances

  • Inflammation Mediators
  • Pentacyclic Triterpenes
  • TRPP Cation Channels
  • Triterpenes
  • polycystic kidney disease 1 protein
  • celastrol