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Molecular Pathway to Protection From Age-Dependent Photoreceptor Degeneration in Mef2 Deficiency.

Abstract Photoreceptor degeneration in the retina is a major cause of blindness in humans. Elucidating mechanisms of degenerative and neuroprotective pathways in photoreceptors should afford identification and development of therapeutic strategies.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title investigative ophthalmology & visual science
Publication Year Start




PMID- 28738418
OWN - NLM
STAT- MEDLINE
DA  - 20170724
DCOM- 20170728
LR  - 20170728
IS  - 1552-5783 (Electronic)
IS  - 0146-0404 (Linking)
VI  - 58
IP  - 9
DP  - 2017 Jul 01
TI  - Molecular Pathway to Protection From Age-Dependent Photoreceptor Degeneration in 
      Mef2 Deficiency.
PG  - 3741-3749
LID - 10.1167/iovs.17-21767 [doi]
AB  - Purpose: Photoreceptor degeneration in the retina is a major cause of blindness
      in humans. Elucidating mechanisms of degenerative and neuroprotective pathways in
      photoreceptors should afford identification and development of therapeutic
      strategies. Methods: We used mouse genetic models and improved methods for
      retinal explant cultures. Retinas were enucleated from Mef2d+/+ and Mef2d-/-
      mice, stained for MEF2 proteins and outer nuclear layer thickness, and assayed
      for apoptotic cells. Chromatin immunoprecipitation (ChIP) assays revealed MEF2
      binding, and RT-qPCR showed levels of transcription factors. We used AAV2 and
      electroporation to express genes in retinal explants and electroretinograms to
      assess photoreceptor functionality. Results: We identify a prosurvival
      MEF2D-PGC1alpha pathway that plays a neuroprotective role in photoreceptors. We
      demonstrate that Mef2d-/- mouse retinas manifest decreased expression of
      PGC1alpha and increased photoreceptor cell loss, resulting in the absence of
      light responses. Molecular repletion of PGC1alpha protects Mef2d-/-
      photoreceptors and preserves light responsivity. Conclusions: These results
      suggest that the MEF2-PGC1alpha cascade may represent a new therapeutic target
      for drugs designed to protect photoreceptors from developmental- and
      age-dependent loss.
FAU - Nagar, Saumya
AU  - Nagar S
AD  - Neuroscience and Aging Research Center and Graduate School of Biomedical
      Sciences, Sanford Burnham Prebys Medical Discovery Institute, La Jolla,
      California, United States.
FAU - Trudler, Dorit
AU  - Trudler D
AD  - Neurodegenerative Disease Center, Scintillon Institute, San Diego, California,
      United States.
FAU - McKercher, Scott R
AU  - McKercher SR
AD  - Neurodegenerative Disease Center, Scintillon Institute, San Diego, California,
      United States.
FAU - Pina-Crespo, Juan
AU  - Pina-Crespo J
AD  - Neuroscience and Aging Research Center and Graduate School of Biomedical
      Sciences, Sanford Burnham Prebys Medical Discovery Institute, La Jolla,
      California, United States.
FAU - Nakanishi, Nobuki
AU  - Nakanishi N
AD  - Neurodegenerative Disease Center, Scintillon Institute, San Diego, California,
      United States.
FAU - Okamoto, Shu-Ichi
AU  - Okamoto SI
AD  - Neurodegenerative Disease Center, Scintillon Institute, San Diego, California,
      United States.
FAU - Lipton, Stuart A
AU  - Lipton SA
AD  - Neuroscience and Aging Research Center and Graduate School of Biomedical
      Sciences, Sanford Burnham Prebys Medical Discovery Institute, La Jolla,
      California, United States 2Neurodegenerative Disease Center, Scintillon
      Institute, San Diego, California, United States 3Department of Neurosciences,
      University of California, San Diego, School of Medicine, La Jolla, California,
      United States 4Department of Molecular Medicine, The Scripps Research Institute, 
      La Jolla, California, United States.
LA  - eng
GR  - DP1 DA041722/DA/NIDA NIH HHS/United States
GR  - P01 HD029587/HD/NICHD NIH HHS/United States
GR  - P30 NS076411/NS/NINDS NIH HHS/United States
GR  - R01 NS086890/NS/NINDS NIH HHS/United States
PT  - Journal Article
PL  - United States
TA  - Invest Ophthalmol Vis Sci
JT  - Investigative ophthalmology & visual science
JID - 7703701
RN  - 0 (MEF2 Transcription Factors)
RN  - 0 (Mef2d protein, mouse)
RN  - 0 (Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha)
RN  - 0 (Ppargc1a protein, mouse)
SB  - IM
MH  - Aging
MH  - Animals
MH  - Apoptosis
MH  - Cell Survival/physiology
MH  - Dependovirus/genetics
MH  - Disease Models, Animal
MH  - Electroporation
MH  - Electroretinography
MH  - Female
MH  - Gene Expression Regulation/*physiology
MH  - Genetic Therapy
MH  - In Situ Nick-End Labeling
MH  - MEF2 Transcription Factors/genetics
MH  - Male
MH  - Mice
MH  - Mice, Inbred C57BL
MH  - Mice, Knockout
MH  - Organ Culture Techniques
MH  - Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha/*genetics
MH  - Photoreceptor Cells, Vertebrate/*physiology
MH  - Real-Time Polymerase Chain Reaction
MH  - Retinal Degeneration/genetics/pathology/*prevention & control
PMC - PMC5525556
EDAT- 2017/07/25 06:00
MHDA- 2017/07/29 06:00
CRDT- 2017/07/25 06:00
AID - 2645904 [pii]
AID - 10.1167/iovs.17-21767 [doi]
PST - ppublish
SO  - Invest Ophthalmol Vis Sci. 2017 Jul 1;58(9):3741-3749. doi:
      10.1167/iovs.17-21767.