Literature DB >> 2591499

Rhodopsin levels and rod-mediated function in Abyssinian cats with hereditary retinal degeneration.

S G Jacobson1, C M Kemp, K Narfström, S E Nilsson.   

Abstract

Abyssinian cats with different stages of a slowly progressive autosomal recessively-inherited retinal degeneration were studied with imaging fundus reflectometry (IFR) and electroretinography (ERG). Maps of the visual pigment distribution were made in an area of retina extending from the posterior pole to the midperiphery. Rhodopsin levels in the midperipheral retina of a 6-month-old affected cat (stage of suspected disease) were reduced about 20% relative to the mean normal value. The same cat, tested at 2.5 yr of age (now moderately advanced stage), showed a 60% reduction. A 3-yr-old affected cat (also moderately advanced) had a reduction in rhodopsin of about 60%. There was no measurable rhodopsin in a 7-yr-old affected cat (advanced stage). Rhodopsin regeneration kinetics at the different stages of disease were found to be similar to those of normal cats. The rod ERG b-wave threshold in the 6-month-old cat was elevated by 0.26 log units; at 2.5 yr of age, the threshold was elevated by 0.48 log units. A 0.34 log units threshold elevation was found in the 3-yr-old cat. There was no detectable ERG in the 7-yr-old cat. The relationship between the rod ERG threshold elevations and the rhodopsin levels was close to that expected if the dysfunction was caused by decreased quantal absorption.

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Year:  1989        PMID: 2591499     DOI: 10.1016/s0014-4835(89)80043-0

Source DB:  PubMed          Journal:  Exp Eye Res        ISSN: 0014-4835            Impact factor:   3.467


  5 in total

1.  Stable transgene expression in rod photoreceptors after recombinant adeno-associated virus-mediated gene transfer to monkey retina.

Authors:  J Bennett; A M Maguire; A V Cideciyan; M Schnell; E Glover; V Anand; T S Aleman; N Chirmule; A R Gupta; Y Huang; G P Gao; W C Nyberg; J Tazelaar; J Hughes; J M Wilson; S G Jacobson
Journal:  Proc Natl Acad Sci U S A       Date:  1999-08-17       Impact factor: 11.205

2.  Amax to scotopic Imax diagnoses feline hereditary rod cone degeneration more efficiently than any other combination of long protocol electroretinogram parameters.

Authors:  Kristina Narfström
Journal:  Doc Ophthalmol       Date:  2008-01-10       Impact factor: 2.379

3.  An early decrease in interphotoreceptor retinoid-binding protein gene expression in Abyssinian cats homozygous for hereditary rod-cone degeneration.

Authors:  B Wiggert; T van Veen; G Kutty; L Lee; J Nickerson; J S Si; S E Nilsson; G J Chader; K Narfström
Journal:  Cell Tissue Res       Date:  1994-11       Impact factor: 5.249

4.  Cone photoreceptors are the main targets for gene therapy of NPHP5 (IQCB1) or NPHP6 (CEP290) blindness: generation of an all-cone Nphp6 hypomorph mouse that mimics the human retinal ciliopathy.

Authors:  Artur V Cideciyan; Rivka A Rachel; Tomas S Aleman; Malgorzata Swider; Sharon B Schwartz; Alexander Sumaroka; Alejandro J Roman; Edwin M Stone; Samuel G Jacobson; Anand Swaroop
Journal:  Hum Mol Genet       Date:  2011-01-18       Impact factor: 6.150

5.  The domestic cat as a large animal model for characterization of disease and therapeutic intervention in hereditary retinal blindness.

Authors:  Kristina Narfström; Koren Holland Deckman; Marilyn Menotti-Raymond
Journal:  J Ophthalmol       Date:  2011-04-14       Impact factor: 1.909

  5 in total

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