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Animal Health Trust
Progressive
retinal atrophy (PRA) is a term for retinal degenerations occuring in many
breeds of dog. Many forms of PRA exist, each form being confined to one or a few
breeds only. The disease results in a degeneration of the light-sensitive
membrane at the back of the eye - the retina - resulting in loss of vision, and
often leading to blindness.
The form of the disease occurring in miniature long-haired dachshunds can be
diagnosed by electroretinography at a few months of age, although obvious
clinical signs take longer to develop. The exact course of the disease can vary
between individual dogs, and some don’t develop symptoms until relatively late
in life.
The disease is caused by a change to a gene involved in sight. This change, or
mutation, occurred spontaneously, but once in the population has been inherited
from generation to generation like any other gene. The mutation upsets the
delicate processes involved in vision and causes the long-term degeneration
seen. This form of PRA shows an autosomal recessive mode of inheritance: two
copies of the defective gene (one inherited from each parent) have to be present
for a dog to be affected by the disease. Individuals with one copy of the
defective gene and one copy of the normal gene - called carriers - show no
symptoms but can pass the defective gene onto their offspring. When two
apparently healthy carriers are crossed, 25% (on average) of the offspring will
be affected by the disease, 25% will be clear and the remaining 50% will be
carriers
There is currently no treatment for the disease. Breeding stock are regularly
checked by eye examination, although this can only pick up affected dogs after
symptoms have developed and will never detect the symptomless carriers.
The gene responsible has been identified at the Animal Health Trust and we have
recently identified the mutation causing the disease. Using the information from
this research, we have developed a DNA test for the disease. This test not only
diagnoses dogs affected with this disease but can also detect those dogs which
are carriers, showing no symptoms of the disease but producing affected pups.
Under most circumstances, there will be a much greater number of carriers than
affected animals in a population. It is important to eliminate such carriers
from a breeding population since they represent a hidden reservoir of the
disease that can produce affected dogs at any time.
The test is available from February 14th, 2005 and information on submitting
samples is given below.
Breeders will be sent results identifying their dog as belonging to one of three
categories:
CLEAR: the dog has 2 copies of the normal gene and will neither develop PRA, nor
pass a copy of the PRA gene to any of its offspring.
CARRIER: the dog has one copy of the normal gene and one copy of the mutant gene
that causes PRA. It will not develop PRA but will pass on the PRA gene to 50%
(on average) of its offspring.
AFFECTED: the dog has two copies of the PRA mutation and is affected with PRA.
It will develop PRA at some stage during its lifetime, assuming it lives to an
appropriate age.
Carriers can still be bred to clear dogs. On average, 50% of such a litter will
be clear and 50% carriers; there can be no affecteds produced from such a
mating. Pups which will be used for breeding can themselves be DNA tested to
determine whether they are clear or carrier.
Samples for testing, either blood (3mls in an EDTA tube) or cheek swabs, should
be sent together with a completed DNA Testing form and a cheque for £60-00 (inc
VAT) for each sample to Genetic Services, Animal Health Trust, Lanwades Park,
Kentford, Newmarket, Suffolk CB8 7UU. DNA testing forms can be downloaded from
our web site (http://www.aht.org.uk). DNA testing forms and cheek swabs can also
be obtained by contacting Vikki Lett 01638 750659 ext 1223 or via e-mail to
vikki.lett@aht.org.uk.
For more
information and a list of Dogs PRA tested
click here.
This will take you to the Longhaired Dachshund Club Website.
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