Elevation of CGMP with normal expression and activity of rod cgmp-pde in photoreceptor degenerate labrador retrievers

Bertel Kommonen, Tarja Kylmä, Robert J. Cohen, John S. Penn, Lars Paulin, Mary Y. Hurwitz, Richard Hurwitz

Research output: Contribution to journalArticle

14 Scopus citations

Abstract

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 β-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.

Original languageEnglish (US)
Pages (from-to)19-28
Number of pages10
JournalOphthalmic Research
Volume28
Issue number1
DOIs
StatePublished - Jan 1 1996

Keywords

  • Animal model
  • Cyclic guanosine 3',5'-monophosphate
  • Cyclic guanosine 3',5'-monophosphate phosphodiesterase
  • Labrador Retriever
  • Retinal dystrophy
  • Ultrastructure

ASJC Scopus subject areas

  • Ophthalmology

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