Juvenile laryngeal paralysis and polyneuropathy (JLPP) is an autosomal recessive, hereditary disease that affects Black Russian Terriers beginning typically around 3 months of age. Affected dogs have difficulty breathing when excited or exercised and may have a change in their bark. As the disease progresses, they develop weakness and loss of coordination in the hind limbs which will eventually progress to affect the front limbs as well. They may also have difficulties swallowing which can result in choking or pneumonia.
EXPLANATION OF JUVENILE LARYNGEAL PARALYSIS & POLYNEUROPATHY RESULTS
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NORMAL
This dog has tested normal (or clear) for the mutation known to cause JLPP. It can only transmit a normal gene to offspring, and can be bred to a dog with any test result without risk of producing affected offspring.
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CARRIER
This dog has tested as a carrier for the mutation known to cause JLPP. There is no evidence at present that these dogs will develop clinical symptoms of JLPP. It may transmit either a normal gene or a mutated gene to potential offspring.
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AFFECTED/AT RISK
This dog has tested as affected for the mutation known to cause JLPP. It is at risk for developing clinical symptoms of JLPP, which generally begin to appear at about 3 months of age. The clinical signs include difficulty breathing, change in bark, weakness and loss of coordination, and difficulty swallowing which can result in choking or pneumonia. There is no effective treatment for this condition, and even with supportive nursing care, these dogs do not live more than a few months after clinical signs begin.
FTA CARD SAMPLE COLLECTION INSTRUCTIONS
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Testing via FTA Cards
FTA Cards are designed to capture and bind DNA samples for future processing. Please follow the directions below to obtain an optimal sample. Directions are also included in the test kit.
Foam tipped applicator swabs are used to collect the sample from the dog, and are then used to apply the sample to the FTA card. The dog’s saliva carries the cheek cells (where the DNA is coming from) from the dog’s cheek to the FTA card. When applying the sample from the swab to the card, enough sample should be collected in order to allow it to soak through the card. Following a successful application, the purple shaded area will fade and change color. The color change should be visible both on the front AND the reverse side of the card. If the card does not change color, it is indicative of a poor sample with insufficient DNA for processing. If the card does not change color, it is OK to reswab the dog and reapply the sample to the same card – however, if doing so, please insure that the swab does not become compromised by coming in contact with DNA from another animal. See the examples below displaying both good and bad collection results.
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GOOD
Following a good collection, the color of the FTA Card card will change where the card has successfully captured DNA. Note the color change on both the front and reverse sides of the card.
Good FrontGood Reverse
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BAD
The following examples are unlikely to yield successful processing results in the lab. Note almost no color change on the front of the card, and no color change at all on the reverse side of the card.
Bad FrontBad Reverse
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Instructions:
Organize first – There should be 1 FTA card (in a small manila envelope), 1 sterile foam tipped applicator swab and 1 test order form containing bar-code and address labels for each individual to be sampled. A second person to handle the dog while you collect the sample and apply it to the card is very useful, and reduces possibility for contamination.
Dogs being swabbed should not have had anything to eat or drink for about an hour prior to collecting the sample. With adult dogs, pick up chew bones, rawhides, and any other toys or treats that contain anything that had been living at some point. If testing young puppies, wake them from a nap, collect the sample, and return them to their mother after you are done. You will need a clean table-top surface to let each card dry safely for about 1 hour. (no wind, and as little dust or other material in the air as possible).
Open the swab. It is VERY IMPORTANT that you do not touch the swab to anything except the mouth of the individual being sampled, and do not touch any of the treated surface of the FTA card (the colored circle and surrounding colored area). Hold the card only by the white untreated area of the card.
Open the dog’s mouth and rub the swab along the inside of the cheek for 5 to 10 seconds. Scrub the cheek with force similar to what you would use if brushing the dog’s teeth so the swab picks up many cheek cells.
Squeeze the swab onto the circle on the card, using pressure to transfer the cells and saliva on the swab to the card. Do not scrub or push so hard that you damage the surface of the card. You can fold the lid of the card over the swab and squeeze it. As it dries, the card will change color from purple to whitish wherever DNA has transferred. You should see this color change and this color change should soak all the way through to the back side of the purple card. If there is no color change, you may need to swab the dog again, making sure to rub the cheek long enough to pick up cells and saliva. If the dog is very dry-mouthed, touch the swab to its tongue once or twice, remove the swab from its mouth and let the dog smack its lips a few times, then try swabbing again. Usually this will cause them to salivate enough to get a good sample.
Allow the card to dry at room temperature for 1 hour. If you have multiple cards to test several dogs, be sure that the cards are not touching each other at any time.
Return each card to its own envelope. Do not put anything else in the envelope with the card. The cards do not need to be refrigerated or frozen – the DNA on them is very stable at room temperature. You do not need to return the foam tipped applicator swab.
Shipping – The cards are very stable and can be sent by regular mail or in the overnight/priority envelopes supplied by shippers. A return shipping label to the University of Missouri is provided on the order form.
OFA'S POLICY ON DNA CLEAR BY PARENTAGE
As a greater number of DNA based disease tests become available, a policy regarding the clearing of offspring out of DNA tested parents has become necessary.
For direct mutant gene tests only, the OFA will issue clearances to untested offspring, if the sire and dam have both been DNA tested “clear,” if the sire and dam’s DNA disease test results have been OFA registered, and if all three (sire/dam/offspring) have been DNA identity profiled and parentage verified. The DNA profile paperwork must be submitted along with a completed OFA DNA-based disease test application. The resulting OFA certification will have a suffix of “CBP” (clear by parentage), indicating that the dog itself was not tested and that the clearance was based on the sire and dam’s test results, and known science at the time. Because of the possibility of new mutations or as of yet undiscovered gene mutations, only first generation offspring will be cleared.
For linkage or marker based tests where a margin of error including both false positives and negatives exists, the OFA will not issue any clearances to untested dogs.
DNA based disease screening is an evolving area. This policy is subject to change by action of the OFA Board of Director.
Inheritance : AUTOSOMAL RECESSIVE trait
Sire
Dam
Offspring
clearclear100% clear
clearcarrier50% clear + 50% carriers
clearaffected100% carriers
carrierclear50% clear + 50% carriers
carriercarrier25% clear + 25% affected + 50% carriers
carrieraffected50% carriers + 50% affected
affectedclear100% carriers
affectedcarrier50% carriers + 50% affected
affectedaffected100% affected
Clear
Genotype: N / N [ Homozygous normal ]
The dog is noncarrier of the mutant gene.
The dog will never develop Juvenile Laryngeal Paralysis & Polyneuropathy (JLPP) and therefore it can be bred to any other dog.
Carrier
Genotype: N / JLPP [ Heterozygous ]
The dog carries one copy of the mutant gene and one copy of the normal gene.
The dog will never develop Juvenile Laryngeal Paralysis & Polyneuropathy (JLPP) but since it carries the mutant gene, it can pass it on to its offspring with the probability of 50%.
Carriers should only be bred to clear dogs.
Avoid breeding carrier to carrier because 25% of their offspring is expected to be affected (see table above)
Affected
Genotype: JLPP / JLPP [ Homozygous mutant ]
The dog carries two copies of the mutant gene and therefore it will pass the mutant gene to its entire offspring.
The dog will develop Juvenile Laryngeal Paralysis & Polyneuropathy (JLPP) and will pass the mutant gene to its entire offspring
