Abstract:
Retinal degeneration is a major cause of blindness in the elderly worldwide. The problem is likely to increase as people live longer lives, imposing a unique challenge to the scientific community to find a cure. In recent years, our understanding of molecular mechanisms behind retinal degenerations has vastly increased. Several key mechanisms have been identified. These include ischemia, oxidative stress, excitotoxicity, autoimmunity, and inflammation. These mechanisms often act in concert with each other, and the final converging point for most of them appears to be apoptosis pathways. Diseases causing retinal degeneration, through their unique pathophysiology, evoke the mechanisms mentioned earlier. This chapter first describes the general mechanisms of retinal degeneration, and then discusses how the disease-specific pathology relates to these mechanisms.
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Abbreviations
- AMPA:
-
amino-3-hydroxy-5-methyl-4-isoxazole propionic acid
- AMD:
-
age-related macular degeneration
- AA:
-
arachidonic acid
- adRP:
-
autosomal dominant retinitis pigmentosa
- bFGF:
-
basic fibroblast growth factor
- CNV:
-
choroidal neovascularization
- CatD:
-
Cathepsin D
- CFH:
-
compliment factor H
- nNOS:
-
Neuronal NOS
- eNOS:
-
endothelial NOS
- FKHR:
-
Forkhead-related transcription factor
- GCL:
-
ganglion cell layer
- GS:
-
glutamine synthetase
- GC:
-
guanylate cyclase
- HIF-1:
-
Hypoxia inducible factor
- HNE:
-
4-hydroxynonenal
- INL:
-
inner nuclear layer
- IPL:
-
inner plexiform layer
- IGF-I:
-
insulin-like growth factor I
- iNOS:
-
inducible nitric oxide synthase
- IAP:
-
Inhibitor of apoptosis proteins
- ICG:
-
indocyanine green
- LCA-1:
-
Leber congenital amaurosis-1
- MAP kinase:
-
mitogen-activated protein kinase
- MD:
-
malondialdehyde
- mGluRs:
-
Metabotropic glutamate receptors
- mtDNA:
-
mitochondrial DNA
- MMPs:
-
matrix metalloproteinases
- NMDA:
-
N-methyl-D-aspartate
- NO:
-
Nitric oxide
- NOS:
-
nitric oxide synthase; nicotinamide
- NMNAT:
-
mononucleotide adenylyl transferase
- OPL:
-
outer plexiform layer
- OH:
-
hydroxyl
- OAT:
-
ornithine-delta-aminotransferase
- PDGF:
-
platelet derived growth factor
- PCNA:
-
proliferating cell nuclear antigen
- PKC:
-
protein kinase C
- PEDF:
-
pigmented epithelium derived factor
- RPE:
-
retinal pigment epithelium
- ROS:
-
Reactive oxygen species
- RP3:
-
X-linked retinitis pigmentosa
- REP-1:
-
Rab escort protein-1
- TNF-[α]:
-
tumor necrosis factor-[α]
- TGF-β:
-
transforming growth factor-β
- TIMPs:
-
tissue inhibitors of metalloproteinases
- TNF-α:
-
tumor necrosis factor-a
- TIMP-3:
-
tissue inhibitor of metalloproteinases-3
- Tyr402His:
-
histidine at amino acid 402
- UbE4b:
-
ubiquitination factor E4b
- VEGF:
-
vascular endothelial growth factor
- XLRD:
-
X-linked retinal degenerations
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Acknowledgments
This work is dedicated to Vani. This work was supported by a generous grant by Gail and Richard Siegal, Gerwin Scholar Award, and Research to Prevent Blindness. The author thanks Dr. Peter A. Netland for his advice and support, Dr. Dianna Johnson and Ozlen Konu for valuable comments on the manuscript and Lee Thompson for help with graphics.
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Sharma, R.K. (2007). Molecular Neurobiology of Retinal Degeneration. In: Lajtha, A., Johnson, D.A. (eds) Handbook of Neurochemistry and Molecular Neurobiology. Springer, New York, NY. https://doi.org/10.1007/978-0-387-30374-1_3
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