A lethal factor or lethal error is an allele of a gene that is fatal in homozygous form before the affected dog is sexually mature. The lethal effect of a lethal factor can occur at different stages of development. The embryo can already die during embryonic development.
Dominant lethal factors are alleles that lead to death even in heterozygous form. Due to this fact, they cannot remain permanently in a population, but arise sporadically through spontaneous mutations. The known lethal factors in our breed are therefore all recessive.

Autosomal recessive means that the disease is inherited regardless of gender and only occurs when the dog's genome has two identical copies of a specific gene. A dog that is only a carrier of the system will NOT fall ill!



Out of a mating of two carriers, theoretically 25% of the offspring are free, 50% of the offspring become carriers and 25% of the offspring are affected by the lethal factor. By mating a not affected dog and a carrier of this mutation, there will theoretically be 50% carriers and 50% free puppies in a litter. Genetic tests thus create the possibility of not mating two carriers with each other and thus not breeding any affected offspring (diseased dogs).

IN GERMANY, LETHAL FACTORS IN SMALL ANIMALS SUCH AS DOGS, CATS, RABBITS AND BIRDS ARE PROHIBITED FROM BREEDING IN ARTICLE 11B OF THE ANIMAL WELFARE ACT (QUALITY BREEDING)

So it is not only ethically unacceptable to breed two carriers, it is also forbidden according to the Animal Welfare Act.

How does the VDH combat hereditary defects in pedigree dog breeding?

The main organization in Germany, to which our breed breeding clubs are subject, has regulated the handling of such defects in the breeding regulations.

Zitat

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1.1 1.1 Association for the German Dog Being -VDH
According to § 4 Breeding Measures No. 1 (VDH Breeding Regulations (as of April 26, 2015)), all breeding measures must aim to combat hereditary defects through suitable breeding programs.

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All four German Belgian Shepherd clubs are subject to these breeding regulations.

Zitat

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The pedigree dog breeding clubs of the VDH are obliged to combat frequently occurring hereditary defects and diseases.... All hereditary diseases with recessive inheritance are an example of the "switching off" of a genetic defect. With this inheritance, dogs only become ill if they have inherited the corresponding defective gene from both their father and their mother. On the other hand, if only one parent inherits the defective gene, the offspring will remain healthy. In these cases, carriers of the genetic defect can be used as breeding dogs if they are important for breeding and are only mated to dogs that do not have this genetic defect. A breeding program achieves the eradication or “elimination” of a genetic defect in a breed through strict criteria for breeding approval and with targeted mating of breeding dogs. Breed clubs are responsible for ensuring that the appropriate breeding programs are followed by their breeders
https://www.vdh.de/presse/das-vdh-guetesiegel/

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Ataxia - an old story

The following quotes come from an article by Volker Riedel, breeding and trail judge of the DMC e.V., which he wrote on the occasion of the C-litter "unter schwarzer Flagge" in 2013. It can be found in full on Mareike's Website

Zitat

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About 30 years ago, when I made my first attempts at breeding Malinois and inevitably ended up in Belgium more and more often, one of the first sentences, I heard there, was:


A good Malinois breeder will either have a river behind the house or a gun in the kennel.

My first Malinois was Kolos de Deux Pottois, a son of G'Bibber. Breeder was Luc Vansteenbrugge, a great connoisseur of the Malinois scene and a visionary breeder. I was very interested at that time and went to Luc very often to listen, learn and understand. It was really still in its infancy. Of course, I saw a lot of litters there. Luc always had 5-8 breeding bitches, spread over farms, whereby the actual kennel was at his mother's. And that's when I saw it for the first time: puppies, about 5-6 weeks old, swaying and with uncoordinated limbs. Not the whole litter of 8 was affected, but only 3 animals. ‘I tried something there’, said Luc, ‘breeded very tight in lines, we'll see.’ There were 2 other litters, all animals without any problems. The next morning, after I had stayed at the hotel, we met at Luc's for breakfast. After that we went to the puppies. In the affected litter the three swaying animals were missing, there were still 5 - all healthy. I looked out the window: Yes, there was a river! ‘You have to make these puppies gone’, said Luc, ‘it never gets better, it just gets worse. Never better!!!’ Two dogs from this litter later became outstanding, in sport and also as sires. Vansteenbrugge simply accepted these losses in order to breed the ideal Malinois for sport and to get a powerful sire. He acted primarily as a breeder. It has always been like this in Malinois breeding: tightly bred dogs had a higher assertiveness in inheritance (with desired and undesired characteristics) and this is how the Malinois has been bred in Belgium for 100 years. Of course, this also led to what I called it at the time, inbreeding depression.

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This "old story" has haunted us up to modern times and was swept under the carpet for many years by breeders and well-known association officials.

Only through Mareike (SDCA1) this topic become public in 2012.
[quote] I fought through the matter at the time, despite massive hostilities, threats from lawyers, etc., and today every breeder and puppy buyer can access information on cerebellar ataxia in Malinois on the Internet. Nobody has to go through the terrible weeks, I went through with my puppies back then. However, I find that nobody really cares. At the time I thought that breeders, puppy buyers and associations would change something when they could read in black and white what catastrophe malinous breeding was heading for - but in reality, little has changed. In large the matings are the same as ever. The dilemma is that the really good dogs come from lines with a high genetic load, and only a few breeders want to go the longer and more difficult route via "fresh blood". I have now completely withdrawn from dog sport and breeding.
Mareike, 2013 " Malinois unter schwarzer Flagge"
quote]

Several breeders have followed her example in the years after her publication and thanks to all of them there are already 4 genetic tests. As it turned out, there is not only one defective gene that leads to the death of puppies and causes ataxia symptoms.

Also, this still doesn't seem to be the end of the road. From the study by the University of Bern on CACA, it can be seen that there are 19 other unexplained cases of puppies with ataxia.

Unfortunately, not all four tests are used by all breeders. In some countries the breeders do not even know about the existence of these genetic defects, kennel clubs dismiss it as "a breeder's problem", although they are obliged by the VDH breeding regulations to control their breeders.

If you, as a breeder, are confronted with a disease in a litter that has not yet been researched, then you being a responsible breeder should contact the University of Bern . If you're hesitant to call them, we're happy to arrange contact for you!


Every puppy buyer should be aware that he can influence that no more sick puppies are born:
DO NOT BUY FROM BREEDERS WHO CANNOT PROVE THAT AT LEAST ONE PARENT HAS BEEN TESTED ‘FREE’ FOR EACH OF THE FOLLOWING SDCA1, SDCA2, CJM AND CACA!



You can find more about this topic in our articles:


CACA - CNS Atrophy with Cerebellar Ataxia

CJM - Cardiomyopathy with juvenile mortality

Spongy Degeneration with Cerebellar Ataxia (SDCA2)

Spongy Degeneration with Cerebellar Ataxia (SDCA1)