X-Linked Progressive Retinal Atrophy 1, XL-PRA1
X-Linked PRA 1 is a retinal disease that causes progressive, non-painful vision loss. The retina contains cells, called photoreceptors, that collect information about light and send signals to the brain. There are two types of photoreceptors: rods, for night vision and movement, and cones, for day vision and color. This type of PRA leads to early loss of rod cells, leading to night blindness before day blindness.
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Signs and symptoms
PRA is a subtle disease and dogs adapt very well to the slow loss of vision. You may notice that your dog is reluctant to go down the stairs, bumps into door frames or corners, or takes a longer time to fetch a toy.
This is a late onset form of PRA with first signs typically between 3-5 years of age. -
Diagnosis
Veterinarians use a focused light to examine the pupils. In affected dogs, the pupils will appear more dilated and slower to contract. Your vet may also use a lens to visualize the retina at the back of the eye to look for changes in the optic nerve or blood vessels. You may be referred to a veterinary ophthalmologist for a definitive diagnosis.
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Treatment
Currently, there is no definitive treatment for PRA. Supplements, including antioxidants, have been proposed for management of the disease, but have not been scientifically proven effective.
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What to do if your dog is at risk
Actions
- Careful monitoring by your veterinarian will be required for the rest of your affected dog's life as secondary complications, including cataracts, can develop.
- With blind dogs, keeping furniture in the same location, making sure they are on a leash in unfamiliar territory, and training them to understand verbal commands are some of the ways to help them at home.
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Genetic Information
This mutation was first described in the Samoyed.
This mutation has a sex-linked recessive inheritance pattern, which means that the gene is on the X chromosome. Females have two X chromosomes and must inherit two abnormal copies to be affected with this disease. Males have only one X chromosome and either have a normal copy of the gene or the mutation.
Gene names:
RPGR (Exon 15) ‐ chr
Inheritance type:
X-linked recessive
Citations:
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Breeds affected
This health condition affects the following breeds
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