+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Inherited retinal dystrophy in the rat

Inherited retinal dystrophy in the rat

Journal of Cell Biology 14: 73-109

Retinal dystrophies, known in man, dog, mouse, and rat, involve progressive loss of photoreceptor cells with onset during or soon after the developmental period. Functional (electroretinogram), chemical (rhodopsin analyses) and morphological (light and electron microscopy) data obtained in the rat indicated two main processes: (a) overproduction of rhodopsin and an associated abnormal lamellar tissue component, (b) progressive loss of photoreceptor cells. The first abnormality recognized was the appearance of swirling sheets or bundles of extracellular lamellae between normally developing retinal rods and pigment epithelium; membrane thickness and spacing resembled that in normal outer segments. Rhodopsin content reached twice normal values, was present in both rods and extracellular lamellae, and was qualitatively normal, judged by absorption maximum and products of bleaching. Photoreceptors attained virtually adult form and ERG function. Then rod inner segments and nuclei began degenerating; the ERG lost sensitivity and showed selective depression of the a-wave at high luminances. Outer segments and lamellae gradually degenerated and rhodopsin content decreased. No phagocytosis was seen, though pigment cells partially dedifferentiated and many migrated through the outer segment-debris zone toward the retina. Eventually photoreceptor cells and the b-wave of the ERG entirely disappeared. Rats kept in darkness retained electrical activity, rhodopsin content, rod structure, and extracellular lamellae longer than litter mates in light.

Please choose payment method:

(PDF emailed within 0-6 h: $19.90)

Accession: 014082724

Download citation: RISBibTeXText

PMID: 13887627

DOI: 10.2307/1604000

Related references

Oculopharyngeal Muscular Dystrophy and Inherited Retinal Dystrophy in Bukhara Jews Due to Linked Mutations in the PABPN1 and NRL Genes. Genetic Testing and Molecular Biomarkers 21(7): 450-453, 2017

Mechanism of retinal ganglion cell loss in inherited retinal dystrophy. Neuroreport 7(12): 1995-1999, 1996

Localization of lysosomal enzymes in retinal pigment epithelium of rats with inherited retinal dystrophy. Investigative Ophthalmology and Visual Science 17(3): 278-288, 1978

Diurnal variations in the electroretinographic c-wave and retinal melatonin content in rats with inherited retinal dystrophy. Documenta Ophthalmologica. Advances in Ophthalmology 79(2): 141-150, 1992

Retinal reductase activity in rats with and without inherited retinal dystrophy. Experimental Eye Research 33(4): 459-462, 1981

Tumor necrosis factor and nitric oxide production by retinal Müller glial cells from rats exhibiting inherited retinal dystrophy. Glia 20(1): 59-69, 1997

Assessment of possible transneuronal changes in the retina of rats with inherited retinal dystrophy: cell size, number, synapses, and axonal transport by retinal ganglion cells. Journal of Comparative Neurology 223(1): 22-34, 1984

Inherited Retinal Dystrophy In The Rat. The Journal of Cell Biology 14(1): 73-109, 1962

Retardation of inherited retinal dystrophy in the rat. Investigative Ophthalmology & Visual Science (SUPPL): 190, 1978

Inherited retinal dystrophy a new animal model. Investigative Ophthalmology & Visual Science (SUPPL): 266, 1978

Inherited retinal dystrophy in Mer knockout mice. Advances in Experimental Medicine and Biology 533: 165-172, 2004

The effect of diet on inherited retinal dystrophy in the rat. Current Eye Research 3(10): 1221-1224, 1984

Therapeutic concepts for inherited retinal dystrophy. Der Ophthalmologe 109(2): 110-111, 2012

An inherited central retinal pigment epithelial dystrophy. Birth Defects Original Article Series 18(6): 281-296, 1982

MiR-204 is responsible for inherited retinal dystrophy associated with ocular coloboma. Proceedings of the National Academy of Sciences of the United States of America 112(25): E3236-E3245, 2015