Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Photoreceptor cell pathology was investigated in an 8-yr-old mixed-breed dog which had displayed visual symptoms of 1 month duration. An electroretinogram detected no light-evoked responses. Light and electron microscopic features showed marked thinning and atrophy of the outer both the tapetal and non-tapetal retina appeared to be involved. In the non-tapetal region, a majority of the rod inner segments were missing, while scattered mitochondria-filled stubby inner segments of cones were readily identified. Inner segments of both rods and cones were observed in the tapetal region. Photoreceptor outer segments were completely absent from the affected retina, and no outer segment debris was observed between the photoreceptor layer and the retinal pigmented epithelium (RPE). Autoradiographic analysis of 3-mm retinal disks from the degenerate retina following incubation with [3H]uridine indicated that only 61% +/- 13 S.D. of the remaining nuclei of rod photoreceptors were undergoing RNA synthesis, whereas more than 99% of cone nuclei incorporated the label. Normal and degenerate retina were also analysed for localization of hydroxyindole-O-methyltransferase (HIOMT)-like immunoreactivity. While the normal retina showed immunoreactivity in both rod and cone photoreceptors with more intense immunoreactivity present in cones, the degenerate retina showed HIOMT-like immunoreactivity only in the remaining cone photoreceptors. The results of this study of idiopathic photoreceptor degeneration of the canine retina suggest that although both photoreceptor types are involved, rods are more severely affected than cones.
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PMID:Idiopathic retinal degeneration in the dog: differential patterns of [3H]uridine incorporation and HIOMT-like immunoreactivity in surviving photoreceptors. 201 60

Glaucoma is an optic neuropathy characterized by progressive degeneration of retinal ganglion cells (RGCs) and visual loss. Although one of the highest risk factors for glaucoma is elevated intraocular pressure (IOP) and reduction in IOP is the only proven treatment, the mechanism of IOP regulation is poorly understood. We report that the P2Y6 receptor is critical for lowering IOP and that ablation of the P2Y6 gene in mice (P2Y6KO) results in hypertensive glaucoma-like optic neuropathy. Topically applied uridine diphosphate, an endogenous selective agonist for the P2Y6 receptor, decreases IOP. The P2Y6 receptor was expressed in nonpigmented epithelial cells of the ciliary body and controlled aqueous humor dynamics. P2Y6KO mice exhibited sustained elevation of IOP, age-dependent damage to the optic nerve, thinning of ganglion cell plus inner plexiform layers, and a reduction of RGC numbers. These changes in P2Y6KO mice were attenuated by an IOP lowering agent. Consistent with RGC damage, visual functions were impaired in middle-aged P2Y6KO mice. We also found that expression and function of P2Y6 receptors in WT mice were significantly reduced by aging, another important risk factor for glaucoma. In summary, our data show that dysfunctional purinergic signaling causes IOP dysregulation, resulting in glaucomatous optic neuropathy.
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PMID:Purinergic dysregulation causes hypertensive glaucoma-like optic neuropathy. 2897 4