Retinal degeneration cells

Arikawa, K., Molday, L. L., Molday, R. S. and Williams, D. S. Localization of peripherine/rds in the disk membranes of cone and rod photoreceptors relationship to disk membrane morphogenesis and retinal degeneration. /. Cell Biol. 116 659-667,1992. 

Bulgakova, N. A., and E. Knust. (2009). The Crumbs complex from epithelial-cell polarity to retinal degeneration. / Cell Sci, Vol.122. pp.2587-2596. 

C1C-6 is a late endosomal chloride transporter. Its disruption in mice led to lysosomal storage disease. C1C-7 is expressed in late endosomes and lysosomes. It needs Ostml as (3-subunit [3]. The disruption of either C1C-7 or Ostml in mice and man leads to severe osteopetrosis, retinal degeneration, and a severe lysosomal storage disease. ClC-7/Ostml is highly expressed in osteoclasts. In these cells, it is inserted together with the proton pump into the specialized plasma membrane ( ruffled border ) that faces the reabsorption lacuna. Osteoclasts are still present in C1C-7 knockout... 

Results in degeneration of retinal ganglion cells and their axons, causing an acute or sub-acute loss of central vision affects predominantly young adult males. Due to one of three point mutations in the ND4, NDl or ND6 subunit genes of complex I. 

Achenbach, S., Liu, Q. and Pierce E.A. (2004) The RPl gene and protein in photoreceptor biology. In Photoreceptor cell biology and inherited retinal degenerations. D.S. Williams, ed. (Singapore World Scientific Publishing Co. Pte. Ltd.), pp. 223-257. 

There is also evidence from animal models that brimonidine may provide neuroprotective properties that could spare retinal ganglion cells and the optic nerve. Using different models to achieve neuronal insult, including mechanical and acute retinal ischemic/reperfu-sion injury, brimonidine appears to protect the optic nerve and retinal ganglion cells from further degeneration. 

Acquired immunodeficiency renders a host much more susceptible to secondary infections, including cytomegalovirus, syphilis, herpes zoster, fungi, hepatitis B, tuberculosis, and toxoplasmosis. HIV invades the tissues of the optic nerve and initiates an immune complex-mediated response that results in an optic neittopathy. The primary HIV infection may be responsible for color vision defects, loss of contrast sensitivity, and visual field defects. HIV infection itself may also cause direct degeneration of retinal ganglion cell axons in the optic nerve without a secondary opportunistic infection. 

Down s syndrome retinal ganglion cell degeneration in glaucoma Cerebral P-amyloid angiopathy ... 

Figure 5.3C shows a cross-section of the retina, but this tissue came from a donor who had lost useful vision due to a retinal degeneration. There is a complete loss of photoreceptor outer segments, the almost complete absence of cells in the ONL (i.e., photoreceptor inner segments). However, the inner retinal cells are preserved in substantial numbers. 

Leber hereditary optic neuropathy (LHON) is the most common mitochondrial disease and the first linked to maternal inheritance through a mutation in the mtDNA. LHON is characterized by bilateral subacute loss of central vision caused by focal degeneration of the retinal ganglion cell layer and of the optic nerve. After initial symptoms, both eyes are usually affected within 6 months. Approximately 50% to 60% of males and only 8% to 32% of females who possess the mtDNA mutation will actually develop this optic neuropathy. Nuclear-encoded factors that affect mtDNA expression, mtDNA products, or mitochondrial metabolism may modify the phenotypic expression of LHON. Genetic coimseling in LHON is complicated in that the amount of mutant mtDNA transmitted by heteroplasmic females cannot be predicted, and testing cannot predict which individuals will develop visual symptoms. ... 

Intravitreally Injected Substances which Rescue Retinal Ganglion Cells from Degeneration... 

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