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Arterial calcification is driven by RAGE in Enpp1-/- mice.

Denise L Cecil1, Robert A Terkeltaub

  • 1Department of Medicine, Rheumatology Section, VA Health Care System/UCSD, San Diego, CA 92161, USA.

Journal of Vascular Research
|November 25, 2010
PubMed
Summary

The receptor for advanced glycation end-products (RAGE) promotes arterial calcification in ENPP1 deficiency by driving ectopic chondrogenesis. Blocking RAGE signaling significantly reduces calcification in Enpp1-/- mice.

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Area of Science:

  • Vascular Biology
  • Mineral Metabolism
  • Chondrogenesis

Background:

  • Generalized arterial calcification of infancy (GACI) is driven by ENPP1 deficiency, leading to reduced inorganic pyrophosphate (PP(i)) and ectopic osteochondral differentiation.
  • The receptor for advanced glycation end-products (RAGE) mediates chondrocyte differentiation and is implicated in atherosclerosis and diabetic complications.
  • Calgranulins, such as S100A11, are ligands for RAGE and can induce chondrocyte differentiation.

Purpose of the Study:

  • To investigate the role of RAGE in ENPP1 deficiency-associated arterial calcification.
  • To determine if RAGE mediates S100A11-induced chondrogenesis and calcification in the context of ENPP1 deficiency.

Main Methods:

  • In vitro studies using mouse aortic explants exposed to exogenous phosphate and alkaline phosphatase.

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  • Assessment of collagen IX/XI expression and calcification in response to S100A11, RAGE, and soluble RAGE (sRAGE).
  • In vivo studies using Enpp1-/- mice with and without Rage knockout to evaluate spontaneous aortic calcification.

    • Main Results:

    • S100A11 induced RAGE-dependent chondrogenic differentiation and calcification in mouse aortic explants, which was inhibited by sRAGE.
    • Enpp1-/- aortic explants showed reduced sRAGE release, increased calcification, and elevated type IX/XI collagen expression, all suppressed by sRAGE or Rage knockout.
    • Rage knockout in Enpp1-/- mice significantly reduced spontaneous aortic calcification.

    Conclusions:

    • RAGE plays a critical role in promoting ectopic chondrogenic differentiation and arterial calcification in ENPP1 deficiency.
    • Reduced sRAGE release in Enpp1-/- aortic explants contributes to RAGE-mediated calcification.
    • Targeting RAGE signaling represents a potential therapeutic strategy for ENPP1 deficiency-associated arterial calcification.