Elevation of cGMP with normal expression and activity of rod cGMP-PDE in photoreceptor degenerate labrador retrievers.

Kommonen B, Kylma T, Cohen RJ, Penn JS, Paulin L, Hurwitz M, Hurwitz RL
Ophthalmic Res. 1996 28 (1): 19-28

PMID: 8726673 · DOI:10.1159/000267869

Cyclic guanosine 3',5'-monophosphate (cGMP) levels were determined in retinas from a strain of Labrador Retrievers with inherited retinal dystrophy manifesting at early stages of retinal differentiation. The cGMP contents of dystrophic retinas of dogs from 1 to 4 months of age (n = 7) were significantly higher (p = 0.001) than in age-matched controls of the same breed (n = 11). Ultrastructure along the vertical retinal meridian was studied in developing retinas and findings were related to those of age-matched wild-type controls of the same breed. Slow central to peripheral progression of degeneration was observed in affected dogs. No differences were found in total cGMP-phosphodiesterase (PDE) activity, in PDE subunit composition as determined by Western blotting of 2-month-old homozygote affected retinas, or in the amino acid sequence deduced from the nucleotide sequence of the PDE beta-subunit as compared to controls. This model of photoreceptor degeneration thus is the first case of an apparent abnormality of cGMP metabolism that is not associated with a defect in the PDE catalytic subunits, and it is also the first reported model not associated with severe developmental abnormalities and rapid degeneration.

MeSH Terms (17)

3',5'-Cyclic-GMP Phosphodiesterases Animals Blotting, Western Cyclic GMP Disease Models, Animal Dog Diseases Dogs Electroretinography Enzyme Activation Microscopy, Electron Nerve Degeneration Photoreceptor Cells Reference Values Retina Retinal Rod Photoreceptor Cells Retinitis Pigmentosa Trypsin

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