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Conditional Müller Cell Ablation Leads to Retinal Iron Accumulation.

Abstract Retinal iron accumulation is observed in a wide range of retinal degenerative diseases, including AMD. Previous work suggests that Müller glial cells may be important mediators of retinal iron transport, distribution, and regulation. A transgenic model of Müller cell loss recently demonstrated that primary Müller cell ablation leads to blood-retinal barrier leakage and photoreceptor degeneration, and it recapitulates clinical features observed in macular telangiectasia type 2 (MacTel2), a rare human disease that features Müller cell loss. We used this mouse model to determine the effect of Müller cell loss on retinal iron homeostasis.
PMID
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Authors

Mayor MeshTerms

Disease Models, Animal

Keywords
Journal Title investigative ophthalmology & visual science
Publication Year Start




PMID- 28846772
OWN - NLM
STAT- MEDLINE
DA  - 20170828
DCOM- 20170904
LR  - 20170904
IS  - 1552-5783 (Electronic)
IS  - 0146-0404 (Linking)
VI  - 58
IP  - 10
DP  - 2017 Aug 01
TI  - Conditional Muller Cell Ablation Leads to Retinal Iron Accumulation.
PG  - 4223-4234
LID - 10.1167/iovs.17-21743 [doi]
AB  - Purpose: Retinal iron accumulation is observed in a wide range of retinal
      degenerative diseases, including AMD. Previous work suggests that Muller glial
      cells may be important mediators of retinal iron transport, distribution, and
      regulation. A transgenic model of Muller cell loss recently demonstrated that
      primary Muller cell ablation leads to blood-retinal barrier leakage and
      photoreceptor degeneration, and it recapitulates clinical features observed in
      macular telangiectasia type 2 (MacTel2), a rare human disease that features
      Muller cell loss. We used this mouse model to determine the effect of Muller cell
      loss on retinal iron homeostasis. Methods: Changes in total retinal iron levels
      after Muller cell ablation were measured using inductively coupled plasma mass
      spectrometry. Corresponding changes in the expression of iron flux and iron
      storage proteins were determined using quantitative PCR, Western analysis, and
      immunohistochemistry. Results: Muller cell loss led to blood-retinal barrier
      breakdown and increased iron levels throughout the neurosensory retina. There
      were corresponding changes in mRNA and/or protein levels of ferritin, transferrin
      receptor, ferroportin, Zip8, and Zip14. There were also increased iron levels
      within the RPE of retinal sections from a patient with MacTel2 and both RPE and
      neurosensory retina of a patient with diabetic retinopathy, which, like MacTel2, 
      causes retinal vascular leakage. Conclusion: This study shows that Muller cells
      and the blood-retinal barrier play pivotal roles in the regulation of retinal
      iron homeostasis. The retinal iron accumulation resulting from blood-retinal
      barrier dysfunction may contribute to retinal degeneration in this model and in
      diseases such as MacTel2 and diabetic retinopathy.
FAU - Baumann, Bailey
AU  - Baumann B
AD  - F.M. Kirby Center for Molecular Ophthalmology, Scheie Eye Institute, Perelman
      School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania,
      United States.
FAU - Sterling, Jacob
AU  - Sterling J
AD  - F.M. Kirby Center for Molecular Ophthalmology, Scheie Eye Institute, Perelman
      School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania,
      United States.
FAU - Song, Ying
AU  - Song Y
AD  - F.M. Kirby Center for Molecular Ophthalmology, Scheie Eye Institute, Perelman
      School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania,
      United States.
FAU - Song, Delu
AU  - Song D
AD  - F.M. Kirby Center for Molecular Ophthalmology, Scheie Eye Institute, Perelman
      School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania,
      United States.
FAU - Fruttiger, Marcus
AU  - Fruttiger M
AD  - Institute of Ophthalmology, University College London, London, United Kingdom.
FAU - Gillies, Mark
AU  - Gillies M
AD  - Save Sight Institute, The University of Sydney, Sydney, Australia.
FAU - Shen, Weiyong
AU  - Shen W
AD  - Save Sight Institute, The University of Sydney, Sydney, Australia.
FAU - Dunaief, Joshua L
AU  - Dunaief JL
AD  - F.M. Kirby Center for Molecular Ophthalmology, Scheie Eye Institute, Perelman
      School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania,
      United States.
LA  - eng
GR  - R01 EY015240/EY/NEI NIH HHS/United States
GR  - T32 EY007035/EY/NEI NIH HHS/United States
PT  - Journal Article
PL  - United States
TA  - Invest Ophthalmol Vis Sci
JT  - Investigative ophthalmology & visual science
JID - 7703701
RN  - 0 (Cation Transport Proteins)
RN  - 0 (RNA, Messenger)
RN  - 0 (Receptors, Transferrin)
RN  - 0 (SLC39A14 protein, mouse)
RN  - 0 (Slc39a8 protein, mouse)
RN  - 0 (metal transporting protein 1)
RN  - 9007-73-2 (Ferritins)
RN  - E1UOL152H7 (Iron)
SB  - IM
MH  - Aged
MH  - Animals
MH  - Blood-Retinal Barrier/metabolism/pathology
MH  - Blotting, Western
MH  - Capillary Permeability
MH  - Cation Transport Proteins/genetics/metabolism
MH  - *Disease Models, Animal
MH  - Ependymoglial Cells/*pathology
MH  - Female
MH  - Ferritins/genetics/metabolism
MH  - Humans
MH  - Immunohistochemistry
MH  - Iron/*metabolism
MH  - Male
MH  - Mass Spectrometry
MH  - Mice
MH  - Mice, Inbred C57BL
MH  - Mice, Inbred CBA
MH  - Mice, Transgenic
MH  - Middle Aged
MH  - RNA, Messenger/genetics
MH  - Real-Time Polymerase Chain Reaction
MH  - Receptors, Transferrin/genetics/metabolism
MH  - Retina/*metabolism
MH  - Retinal Telangiectasis/genetics/*metabolism
PMC - PMC5574447
EDAT- 2017/08/29 06:00
MHDA- 2017/09/05 06:00
CRDT- 2017/08/29 06:00
AID - 2652621 [pii]
AID - 10.1167/iovs.17-21743 [doi]
PST - ppublish
SO  - Invest Ophthalmol Vis Sci. 2017 Aug 1;58(10):4223-4234. doi:
      10.1167/iovs.17-21743.