https://www.cell.com/cell/pdf/S0092-8674(00)80602-9.pdf

Insights into the Function of Rim Protein in Photoreceptors and Etiology of Stargardt's Disease from the Phenotype in abcr Knockout Mice

PURPOSE. TO examine the protective effect of a number of survival factors on degenerating photoreceptors in mutant mice with naturally occurring inherited retinal degenerations, including retinal degeneration (rd/rd), retinal degeneration slow (rds/rds), nervous (nr/nr), and Purkinje cell degeneration (pcd/pcd), in three different forms of mutant rhodopsin transgenic mice and in light damage in albino mice. METHODS. Various survival factors were injected intravitreally into one eye of mice at or soon after the beginning of photoreceptor degeneration, with the opposite eye serving as the control, and the eyes were examined histologically at later ages. The survival factors included brain-derived neurotrophic factor (BDNF), neurotrophin-3, neurotrophin-4, ciliary neurotrophic factor (CNTF), Axokine' M (a mutein of CNTF), leukemia inhibitory factor, basic fibroblast growth factor, and nerve growth factor and insulin-like growth factor II, either alone or in various combinations. RESULTS. Photoreceptor degeneration was slowed in rd/rd and nr/nr mutant mice and in Q344ter mutant rhodopsin mice by certain forms of CNTF; the degeneration in Q344ter mice was slowed by Axokine and by leukemia inhibitory factor; and the degeneration in a few nr/nr mice was slowed by BDNF. The other agents were ineffective in these mice, and none of the agents were effective in the other mutants and other mutant rhodopsin transgenic mice. However, light damage experiments that compared agent effectiveness in albino mice versus rats suggested a significant delivery problem with the very small mouse eye, thereby making the interpretation of negative findings equivocal in mutant mice. Basic fibroblast growth factor failed to protect the mouse retina from the damaging effects of constant light, whereas it showed a strong protective effect in the rat, indicating an important species difference. CONCLUSIONS. The slowing of degeneration in the rd/rd and Q344ter mutant mice demonstrated that intraocularly injected survival factors can protect photoreceptors from degenerating in animal models with the same or similar genetic defects as those in human inherited retinal degenerations. (Invest Opbthalmol Vis Sci. 1998;39:592-602)

Protection of mouse photoreceptors by survival factors in retinal degenerations.

Retinal light damage: mechanisms and protection.

Assessing retinal function with the multifocal technique

The molecular and cellular basis of rhodopsin retinitis pigmentosa reveals potential strategies for therapy

11 Metalloproteinases (MPs) present in the interphotoreceptor matrix (IPM) may play a role in both the maintenance of normal retinal homeostasis and the progression of various degenerative retinal diseases. We wished to extend our previous studies of these enzymes and begin their characterization by first obtaining them in purified form. IPM was obtained from fresh bovine eyes. The material was fractionated by a combination of gel filtration, DEAE ion exchange and gelatinaffinity chromatographies. Fractions were monitored by SDS-PAGE carried out on both plain gels and those preloaded with gelatin or casein (zymograms) and by quantitative analysis for enzyme activity Combination of the procedures described above provided a partial fractionation of the MPs as detected by zymography. A previously unrecognized 52 kDa caseinase was also revealed, possibly identical with MMP-3 (stromelysin).

Degenerative diseases of the retina

New insights into retinal degenerative diseases

Retinal Degeneration: Clinical And Laboratory Applications

Retinal Degenerations: Mechanisms And Experimental Therapy

Inherited and environmentally induced retinal degenerations : proceedings of the International Symposium on Retinal Degenerations, held in San Francisco, California, September 2-3, 1988

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