# NCL and other Genetic diseases



## Jason Moore (May 3, 2009)

I have been getting aggrivated at the lack of testing being done for NCL in american bulldogs. When you have a disease that will limit a dogs life to 5 years and not a happy 5 years and is completely and easily avoidable it just pisses me off. I don't know what all diseases there are out there but that this is one of the more common that seems to be out there in AB's. How can you call your self a breeder and not care enough about the breed to make sure that the dogs you sell will not be affected or a carrier of this and other diseases. IMO this is just as bad or worse than breeding a dog with hip displaysia. I think any dog found to be affected or a carrier should be spayed or neutered. Just my opinion.


----------



## Julie Ann Alvarez (Aug 4, 2007)

Jason you need to check out the True Grit board for more info. This is the original article in the ABA Review.

Hi fellow bulldoggers. Last issue I wrote an article about Neuronal Ceroid Lipofuscinosis for the American Bulldog Review. When I wrote the article, I was feeling very positive that we had the disease, for the most part, under control. As it turns out, it appears I was being unduly optimistic. Here are some figures I’ve received, courtesy of Liz Hansen at the University of Missouri, enumerating the number of dogs so far tested, and the results of those tests. 

As of 11/22/05, 178 dogs have been tested. 60 dogs “carriers”. 104 dogs “clear”. 14 dogs “affected”. There are 20 more tests pending. This is just the beginning, so it’s clear that this is a major issue already.

Basically a clear dog bred to a carrier will, on average, produce 50% clear pups and 50% carriers, with no affected pups. 2 carriers bred together will produce 50% carriers, 25% affected and 25% clear pups, on average. With a little thought you can see how just a few breedings, even of carrier to clear, will produce a lot of potential problems for the breed. 

The test now looks for a couple of specific genes, and if either of those genes are present, then the dog is either a carrier, or is (or will soon be), affected with the disease. This test no longer looks for “marker” genes to determine a dog’s status (somewhere around 70-80% accurate), but now looks for the actual problem gene (100% accurate). I’ve been criticized lately for making a big deal out of this disorder. I’ve also been told that this is no different than hip dysplasia, or ichthyosis, or any of the other genetic problems that affect American Bulldogs. One person actually told me that because a certain dog has never thrown an affected pup, there’s no reason that he or she should be tested even though he’s out of known carriers, and has produced many offspring. While my first instinct was to call people who think this way “money grubbing morons”, maybe it’s my fault for not making clear the problems with the disease.

First of all, affected dogs seem to show no signs of a problem until they are around 18 months old. This means the owner has quite possibly spent a considerable amount of money, time, and effort feeding, training, and maybe even showing the dog with no clue of the impending problems. This doesn’t even begin to take into account the emotional attachment or bond that is a part of any good owner/dog relationship. Just image how you would feel if your favourite dog, one you spend a great deal of time and effort on, suddenly started to look like it has severe hip dysplasia (showing limited control over its back legs.) The dog, while it doesn’t appear to be in any pain, has a difficult time walking, and even standing on a slippery surface sees the dog start to do the splits with its hind legs, without even knowing or feeling them move. Many vets, just seeing the dog in the early stages, will guess that it has hip dysplasia. Within the next 1 to 3 years, the dog won’t even be able to keep from falling into its own faeces or urine. When it walks it tends to stagger and move haphazardly, especially if it must go down a flight of stairs or over uneven terrain. When it runs, its rear hops and bounces around showing very little control. Normally, by the time the dog is 5 years old, it’s been euthanised, as just standing is very difficult, much less trying to spasmodically walk. With hip dysplasia, the average couch potato dog can live a reasonably normal life, even if it has to be on pain killers like rimadyl. Also, except for severely dysplastic dogs, most dogs won’t even show signs of a problem until later in life. With ichthyosis, puppies are seen to have skin problems before they’re 2 weeks old, and can be euthanised before there is a large emotional or financial investment in them. The pup is never sold, and in fact never leaves the breeders’ yard. While I’m not in any way trivializing these very serious genetic problems, they seem much easier for the average pet owner to deal with than NCL. 

And finally, as to which dogs to test, let me say that within the next few years it is likely that many people will be demanding that most dogs are certified “clear” of NCL regardless of their pedigrees. I say this because some of the best looking and tempermented dogs in the country will be found to be carriers, and if 2 carriers are bred together, 25% of the pups will become “affected”, and 2/3 of the remaining pups will be carriers, capable of passing the defective gene on. While I don’t want this to become a witch-hunt, breeders and owners need to know the most influential carriers of the disorder. As I’ve said before, my dogs (though not particularly influential in the breed) Diesel, GrCh 2 Spots None the Richer, (and now I know) Domani and Gryffyndogge are all carriers. In Matt Boyd’s line Moleque and High Jumping Mikie were the most influential dogs that were carriers. Greg Souza recently informed my wife Victoria, along with many people at our show, that Koa and Matias are both carriers. Close to 10 years ago there was a very special breeding that produced Rob Boyd’s “Hammer” as well as several other very nice looking dogs. While I do not know if “Hammer” was a carrier, one of his littermates definitely was, and so he and any of his littermates have a 50% chance of being carriers. (I only mention this because my dog 2 Spots is a Grandson of “Romero’s Little Moose”, a carrier from the above litter). All dogs with any of these dogs in their pedigree need to be tested before breeding! In my opinion, (and I know I’m going to receive a lot of heat for this!!) this does not mean that a carrier cannot be bred. However, it does mean that it should be an exceptional dog, it must not be bred to another carrier, and that if bred to a clear dog, the breeder has a responsibility to test the puppies to determine which are carriers and which are clear before placing or selling them, along with documentation showing the status of each pup. I believe that to breed a carrier is a serious breeder responsibility!!!! Hopefully within 3 or 4 generations there will be no reason to breed to any carriers. 

Thanks to the University of Missouri, there is a $40 DNA test (a simple blood test) available to determine whether or not a dog is a carrier of the defective gene, or will be affected by the disease. For puppies, one can send in their dew claws, therefore getting results before they’re ready to go to their new homes. There is no way of telling whether or not a dog is a carrier without testing for it. There is virtually no way of telling if a pup will be affected by the disease before he’s around 18 months of age without testing for it. The website for info on testing at the University of Missouri is: http://www.caninegeneticdiseases.net/CL_site/mainCL.htm A link to the website is also available on Matt and Suzie Boyd’s site http://www.boydsbulldogs.com/ There is also a site dedicated to Border Collies in Australia with NCL. While the symptoms of the disease manifest themselves differently, the mode of inheritance chart at the bottom of the site is the same for our dogs. http://www.mybcsite.com/bccnswwebfiles/clinformation.htm#Genetic Inheritance 

If you have any questions about NCL, I’m always happy to dispense what information I have. My e-mail is [email protected]. Let’s not rag on each other over dogs that are found to be carriers, but work together and share testing results so we can all make intelligent, informed decisions about our breedings. Knowledge and openness will quickly help us eradicate this disease before it assumes epidemic proportions. Ed NeSmith


Julie


----------



## Julie Ann Alvarez (Aug 4, 2007)

Here is another link.

http://www.caninegeneticdiseases.net/CL_site/mainCL.htm


----------



## Julie Ann Alvarez (Aug 4, 2007)

OFA has started a data base but it only works if people file.

http://www.offa.org/results.html?num=&registrar=&namecontains=N&part=&breed%5B%5D=AMB&breedlist=ALL&variety%5B%5D=&sex=&birthday_start_month=&birthday_start_year=&birthday_end_month=&birthday_end_year=&birthday=&regand=N&regcode%5B%5D=NCL&rptdte_start_month=&rptdte_start_year=&rptdte_end_month=&rptdte_end_year=&rptdte=&submit=Begin+Search


----------



## Jason Moore (May 3, 2009)

Again thanks for all your help Julie. I would like to place you on my buddy list if thats ok with you?


----------



## Julie Ann Alvarez (Aug 4, 2007)

Sure, I would love to be your buddy:lol: 

Hey tell your friend our club has 3 Zidane off spring (all very nice working dogs). 


Julie


----------

