Purchase this article with an account.
Delu Song, Liangliang Zhao, Yafeng Li, Majda Hadziahmetovic, Ying Song, Joshua L Dunaief; The oral iron chelator deferiprone protects against iron overload-induced retinal degeneration in Hepcidin knockout mice. Invest. Ophthalmol. Vis. Sci. 2014;55(13):415.
Download citation file:
© ARVO (1962-2015); The Authors (2016-present)
To investigate the retinal protective effects of the oral iron chelator deferiprone (DFP) in Hepcidin (Hepc) knockout mice with age-dependent retinal iron accumulation and some pathologic features of AMD.
Retinas from Hepc knockout mice, with or without DFP in drinking water, were analyzed by fundus imaging, electroretinography (ERG), histology, immunofluorescence and quantitative PCR to investigate the protective effect of DFP on retinal degeneration.
In Hepc knockout mice, DFP protected against retinal degeneration indicated by fundus imaging. ERG rod-a, -b and cone-b wave amplitudes were significantly higher in DFP treated mice. Plastic sections showed photoreceptor and retinal pigment epithelial (RPE) cells were preserved in Hepc ko mice treated with DFP. The mRNA level of oxidative stress-related gene heme oxygenase-1 was significantly decreased in DFP treated Hpec ko retinas. Consistent with histologic results, the mRNA level of rhodopsin was significantly higher in retinas treated with DFP. Immunolabeling with L-ferritin antibody showed significantly decreased signals in RPE and inner/outer segments. Additionally, autofluorescense in the RPE layer in cryosections was significantly diminished by DFP which is consistent with the fundus images.
The oral iron chelator DFP diminished retinal iron levels and oxidative stress, providing significant protection against retinal degeneration in Hepc ko mice. This indicates that iron chelation could be a useful treatment for retinal disease involving iron overload or oxidative stress.
This PDF is available to Subscribers Only