Progressive retinal atrophy

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The progressive retinal atrophy (PRA) is a slowly progressive death of the retina of dogs and - less frequently - cats . It is a hereditary disease that ultimately leads to blindness in both eyes. The degenerative processes begin in the area of ​​the photoreceptors and extend to the entire retina in the course of the disease. Despite the similar clinical appearance, the symptoms can have a number of different causes.

Progressive retinal atrophy corresponds to retinopathia pigmentosa in humans.

distribution

The disease affects a wide variety of dog breeds and their mixed breeds . It was demonstrated in minke and means Poodle , American Cocker , full English Cocker , Portuguese Water Dog , Labrador Retriever , Samoyed , English Setter , Rauhaar- and long-haired dachshund , Tibetan Terrier , Tibetan Spaniel , Akita Inu , Siberian Husky , Afghan , Australian Cattle Dog , Malinois , St. Bernard , Border Collie , Cavalier King Charles Spaniel , Chesapeake Bay Retriever , Collie , English Springer Spaniel , Entlebucher Sennenhund , Golden Retriever , Irish Setter , Irish Terrier , Maltese , Mastiff , Nova Scotia Duck Tolling Retriever , Giant Schnauzer , Rottweiler , Saluki , Sloughi , Great Pyrenees , Bolognese , Newfoundland Dogs , PON , Saarloos Wolfdogs , Scottish Terriers , Schapendoes , Do Khyi , Sheltie , Yorkshire Terriers , Miniature Spitz and crosses between Spaniels and Poodles.

In cats , the Somali and Abyssinian breeds are affected.

root cause

The cause is in most cases in an autosomal - recessive inherited genetic defect. Mastiffs describe a dominantly inherited form. Huskies and Samoyed form a variant bound to the X chromosome .

treatment

To date, there are no medications or surgical measures to cure the PRA. Dogs with PRA almost always go blind. In the X-chromosome-based form of PRA, gene therapy was able to stop the increasing loss of photoreceptors, but this treatment is still at the experimental stage.

Genetic evidence

Genetic testing to detect PRA is available for a number of dog breeds. These include Irish Setters, Welsh Corgi, Sloughi, Bull Terrier, Mastiff, and Bullmastiff.

Individual evidence

  1. ↑ Cynthia M. Kahn et al: Inherited Retinopathies. In: The Merck Veterinary Manual. 9th edition. 2005.
  2. ↑ a b B. Nell, I. Walde: eyes. In: EG Grünbaum, E. Schimke: Clinic of dog diseases. Enke, Stuttgart 2007, ISBN 3-8304-1021-2 , p. 329.
  3. ↑ G. Dekomien, M. Runte et al .: Generalized progressive retinal atrophy of Sloughi dogs is due to an 8-bp insertion in exon 21 of the PDE6B gene. In: Cytogenetic and Genome Research. 90, 2000, pp. 261-267, doi: 10.1159 / 000056785 .
  4. ↑ Gabriele Dekomien, Joerg T Epplen: Analysis of PDE6D and PDE6G genes for generalized progressive retinal atrophy (gPRA) mutations in dogs. In: Genetics Selection Evolution. 35, 2003, p. 445, doi: 10.1051 / gse: 2003033 .
  5. ↑ http://www.laboklin.de/index.php?link=labogen/pages/html/de/erbkrankheiten/hund/hund_progressive_retinaatrophie-prcdpra.html
  6. ↑ laboklin.de ( Memento from November 19, 2010 in the Internet Archive )
  7. ↑ SM Petersen-Jones: Progressive Retinal Atrophy: An Overview. In: Proceedings of the 28th World Congress of the World Small Animal Veterinary Association. 2003.
  8. Jump up ↑ WA Beltran, AV Cideciyan, S. Iwabe, M. Swider, MS Kosyk, K. McDaid, I. Martynyuk, GS Ying, J. Shaffer, WT Deng, SL Boye, AS Lewin, WW Hauswirth, SG Jacobson, GD Aguirre : Successful arrest of photoreceptor and vision loss expands the therapeutic window of retinal gene therapy to later stages of disease. In: Proceedings of the National Academy of Sciences . Volume 112, number 43, October 2015, pp. E5844 – E5853, doi : 10.1073 / pnas.1509914112 , PMID 26460017 , PMC 4629324 (free full text).
  9. ↑ A. Kehl, P. Kühnlein, I. Langbein-Detsch, E. Müller: Genetic tests in veterinary medicine: What is possible today? In: veterinary mirror. 2007; 17 (1), pp. 28-30. ( online ( memento of August 21, 2014 in the Internet Archive ); PDF; 852 kB) doi: 10.1055 / s-0029-1233557

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