April 2009
Volume 50, Issue 13
Free
ARVO Annual Meeting Abstract  |   April 2009
Hepcidin Deficient Mice Have an Age-Related Retinal Iron Accumulation With RPE Hypertrophy/Autofluorescence
Author Affiliations & Notes
  • M. Hadziahmetovic
    FM Kirby Ctr, Scheie Eye Insitute/Univ of Penn, Philadelphia, Pennsylvania
  • Y. Song
    FM Kirby Ctr, Scheie Eye Insitute/Univ of Penn, Philadelphia, Pennsylvania
  • S. Grieco
    FM Kirby Ctr, Scheie Eye Insitute/Univ of Penn, Philadelphia, Pennsylvania
  • S. Vaulont
    Institut Cochin, Université Paris Descartes, Paris, France
  • J. L. Dunaief
    FM Kirby Ctr, Scheie Eye Insitute/Univ of Penn, Philadelphia, Pennsylvania
  • Footnotes
    Commercial Relationships  M. Hadziahmetovic, None; Y. Song, None; S. Grieco, None; S. Vaulont, None; J.L. Dunaief, None.
  • Footnotes
    Support  Research to Prevent Blindness (William and Mary Greve Scholar Award), International Retina Research Foundation Alston Callahan, MD Award, NIH EY015240, Macula Vision Research Foundation
Investigative Ophthalmology & Visual Science April 2009, Vol.50, 775. doi:
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    • Get Citation

      M. Hadziahmetovic, Y. Song, S. Grieco, S. Vaulont, J. L. Dunaief; Hepcidin Deficient Mice Have an Age-Related Retinal Iron Accumulation With RPE Hypertrophy/Autofluorescence. Invest. Ophthalmol. Vis. Sci. 2009;50(13):775.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose: : Iron is an essential cofactor in heme and nonheme-containing enzymes, but also a potent generator of damaging free radicals that can cause oxidative stress. Consistent with a need of the organism for precise iron regulation, the eye also requires tight iron regulation. Hepcidin, a 25aa hormone, is an important regulator of systemic iron homeostasis, but its function within the eye is unclear. Robust hepcidin expression within the retina (Müller, photoreceptor and RPE cells {Gnana-Prakasam JP et al. Biochem J. 2008 Apr 1}) suggests that the retina produces hepcidin for regulation of local iron homeostasis.In this study, to better understand the importance of hepcidin in retinal iron homeostasis, mice with targeted disruption of the hepcidin 1 gene (Hepc1-/- mice) were used.

Methods: : Expression of Hepcidin in normal mouse retina was assessed by Northern Blotting. Morphologic analysis and histochemical iron detection by Perls’ staining was performed on retina sections from Hepc1-/- miceand control mice. Immunofluorescence was performed with antibodies detecting L-ferritin. Tissue iron levels were measured by atomic absorption spectrophotometry.

Results: : We found that hepcidin deficiency leads to similar retinal changes as those observed in mice deficient in both multicopper ferroxidases, Cp and Heph (double KO mice), including age-related retinal iron accumulation, increased iron storage protein ferritin, and ultimately retinal degeneration.

Conclusions: : Targeted disruption of the hepcidin 1 gene led to some AMD-like features (RPE hypertrophy and autofluorescence with photoreceptor death), suggesting an essential role of hepcidin in retinal iron regulation.

Keywords: age-related macular degeneration • retinal pigment epithelium 
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