The junctional epidermolysis bullosa (E.B.J.)
What to do?
The junctional epidermolysis bullosa (EBJ) currently affecting our breed, it is a serious genetic disease manifested in puppies before weaning at approximately fourth week age, and translated initially by ulcers in the ears and pads; affected pups were very little chance of survival and must be euthanized. This disease its mode of inheritance have been demonstrated in the gsp by the work of Dr. Guaguère (59 Lomme,France). It is a genetic disease,transmitted by "asymptomatic carriers" themselves healthy of the disease, but carriers of the gene recessive and transmit to their descendants. Several large kennels have experienced in the early 90s, and through sense of responsibility of those breeders who have immediately taken the necessary steps, a genetic test has been developed through Dr. Guaguère, mentioned above, teams INSERM Nice Labogena , which manufactures and markets the test today.
Transmission Mode
The junctional epidermolysis bullosa is transmitted hereditarily according to Mendel's laws, through a recessive gene, that is to say a gene "Hidden" that only DNA testing can detect. So it is transmitted the same way that the mottled coat, for example, who is also an recessive trait . This explains for example, that two dogs unicolored can produce ticked puppies , and two healthy dogs can produce puppies suffering from E. B.J. So that the disease occurs within a scope, it is necessary that both parents are carriers of the disease. In this case, we will have Statistically 25% of ill subjects, 50% individuals infected who can transmit the disease in turn, and 25% of healthy subjects, not carriers. More may be serious for the future of the race: if we crosses one carrier dog, male or female, with a non carrier dog, the disease does not declare - and will be ignored - but they produce 50% of new carriers which in turn will disseminate the disease, without their producers or owners are aware of it! Note that all tests performed on complete litters from a parent carrier confirmed the rate of 50% new carrier subjects from the litter! No risk, however if we cross the subjects non carriers. Through the test, it will be possible now to select non carrier dogs to safely use in breeding, even if ancestors come from carriers, which will keep most of the achievements of our breeders. We can not be sufficiently grateful to all those who acted for that screening .