wildairbc Posted September 8, 2004 Report Share Posted September 8, 2004 PRESS RELEASE Research team at University of NSW identifies gene for nerve degenerative disease in dogs. Dr Alan Wilton and Scott Melville from the School of Biotechnology and Biomolecular Sciences have identified the mutation that causes ceroid lipofuscinosis, more commonly known as CL, which is major problem in border collie dogs. Affected animals suffer slow neural degeneration from about 15 months which leads to behavioural and motor problems inevitably leading to death within a year. The discovery will eventually allow DNA testing for the disease. Cheap and effective methods of detecting asymptomatic carriers of the gene defect are currently being developed and will be available within 6 months. The test will allow the eliminate of a major genetic problem from the breed by DNA testing and selective breeding. With a simple DNA test it will even be possible for breeders to use a carrier in a mating and then identify which of the pups are free of the defect and can be used in further breeding, and which are carriers and would better kept as pets. The disease in dogs is a good model for the human form of the disease, which is known as Batten disease. It could be useful in understanding the cause of the disease and in the development of treatment. The research has been supported by grants from Australian Research Council SPIRT program with the Border Collie Club of NSW as partners, Canine Research Foundation, Batten Disease Support and Research Association, bequest from Alice McDonald (Cairns) as well as contributions from Border Collie Clubs both in funding and access to samples. Research in the lab continues into causes of other genetic disorders such as Trapped Neutrophil Syndrome (TNS), in which neutrophils get stuck in the bone marrow leaving pups immunodeficient and very susceptible to gastric infections and slow development. The release of the dog genome sequence in July makes the discovery of such defects much easier than it was. All dog breeds have their own unique genetic defects as a result of the breeding structure of extensive use of champion dogs followed by matings between relatives. This inbreeding reveals any underlying genetic defects which are occur naturally in us all. The Border Collie Clubs are to praised for recognising that they had a problem in the breed, and for bringing it out in the open where it could be dealt with, instead of pretending it did not exist, which is the case in most breeds of domestic animal breeds. Quote Link to comment Share on other sites More sharing options...
Bill Fosher Posted September 9, 2004 Report Share Posted September 9, 2004 It should be pointed out that this is not a problem outside of Australian show lines. It's great the gene has been identified. Quote Link to comment Share on other sites More sharing options...
karrider Posted September 9, 2004 Report Share Posted September 9, 2004 Just a tad complacent to say it hasn't been found outside Australian show lines isn't it???? There has been a suspected case here in the UK, the dogs owners did not want an autopsy so it was not completely confirmed, the dog in question was from ISDS lines only. CL can also be used as a model for Battens disease which affects children who rarely survive beyond 7 years of age. Also CL is not just a "show" illness in BC's it has been identiefied in other breeds, Red Setters, Dachunds and Chihuahua to name a few, there are more,just can't think of them off the top of my head Karin Quote Link to comment Share on other sites More sharing options...
Bill Fosher Posted September 9, 2004 Report Share Posted September 9, 2004 Within the Border collie breed, there has never been a case confirmed that wasn't traceable to Australian show lines. That's not complacency, that's simply a fact. Quote Link to comment Share on other sites More sharing options...
karrider Posted September 9, 2004 Report Share Posted September 9, 2004 What are the show lines based on........Original working lines, The small gene pool in Australia at the time is probably responsible for doubling up on the CL, but it had to originate from somewhere in the first place. There was a case of CL in Texas several years ago,the dog was from Unknown parentage,but assumed to be farm bred, I do not know any further details other than that. Karin Quote Link to comment Share on other sites More sharing options...
juliepoudrier Posted September 9, 2004 Report Share Posted September 9, 2004 Karin, Your attempt at trying to lay the blame for CL within the working bred border collies is completely ridiculous, and, I might add, a typical tactic of the KC types who refuse to take responibility for their role in concentrating genetic problems through their breeding practices. Obviously the gene exists in the border collie, else it couldn't have been concentrated in Australian show lines. I repeat, the point Bill was making, correctly, is that the show line breeding in Australia is what concentrated the gene in the show line border collies. That doesn't mean that a very occasional incidence won't occur in the working population of dogs; it simply means that the disease is a *problem* in mainly Australian show lines. Just like diseases that are a real problem in some closed human communities are not a real problem in the human population in general. J. Quote Link to comment Share on other sites More sharing options...
SoloRiver Posted September 9, 2004 Report Share Posted September 9, 2004 The distribution of the disorder would seem to indicate that it arose from a rare mutation and became established in Australasian conformation lines through founder effect. It could have happened in any inbred population of dogs but in this case it happened to have happened in Barbie Collies. Perhaps there are one or two working Border Collies out there suffering from CL, but that doesn't make it a "Border Collie problem," any more than an isolated case of Fanconi in Border Collies (I can dig up the ref if you're interested) makes Fanconi a "Border Collie problem." CL is a Barbie Collie problem. That said, I too am glad the gene has been found. I don't feel like it has much to do with Border Collies, but it is a good thing. Quote Link to comment Share on other sites More sharing options...
BigD Posted September 9, 2004 Report Share Posted September 9, 2004 I'm actually glad the gene has been found because of the situation here in Hawaii. The bulk of our Border Collies have some tie to Australia due to the quarentine laws of Hawaii (now changed). However, there are many people on these islands that have BorderFame dogs that plan to breed. These dogs are sport dogs first, confirmation dogs second and hobby herding dogs a far third. (I think they put the dogs on stock just to pacify those of us out here that frown upon them.) Also, all the dogs from our puppy-mill of a pet store are flown in from Australia. (with papers! how nice) And of course, these dogs are not sold on a spay/neuter contract - nor are the BorderFame dogs - so these people like to make some extra cash and now we've got lots of dogs from Australia lines being bred to Australian lines. There are a few folks on the islands the bring dogs in from the UK for breeding. It's rare, but that's where I got my pup from. There is also someone here that does AI in her breeding from the mainland. Luckily, these folks do NOT bred their dogs to anyone on island, but that may change with the few borderfame fluffy barbie collies. Not sure. I'm just glad it's been found as it will probably pop up here before it gets to the mainland. Denise Quote Link to comment Share on other sites More sharing options...
karrider Posted September 9, 2004 Report Share Posted September 9, 2004 Originally posted by juliepoudrier:[QB] Karin, Your attempt at trying to lay the blame for CL within the working bred border collies is completely ridiculous, and, I might add, a typical tactic of the KC types who refuse to take responibility for their role in concentrating genetic problems through their breeding practices. Obviously the gene exists in the border collie, else it couldn't have been concentrated in Australian show lines. I am not at all interested in laying blame anywhere, just pointing out that a Australian lines where originally bred from working Border Collies, and if you read my post I did also say that the small gene pool was most likely the reason the gene reared its ugly head in the first place, nothing ridiculous in that I think. Karin Quote Link to comment Share on other sites More sharing options...
Darien Posted September 12, 2004 Report Share Posted September 12, 2004 I thought long and hard before answering this, it's hard to recall sad and bitter memories, but I will make this as short as possible so as not to bore you all. In 1991 I lost my beloved Cassie to CL and let me assure you at the time, the BC Club of NSW was of no help to me at all and refused to name the TOP breeder of BC's of the time! As far as I am aware, Bill is correct, it is a disease that has mainly been found in Australian Show bred lines and some of these breeders are still breeding!!! If you are truly interested Karin, I can give the breed line that a lot of theses cases have been found in. With the help of the NSW University of Vet Science, I did a lot of research at the time and still have some of the papers. The BC Club have the rest All I cam say is, Thank God something positive is being done. I hope no one else ever goes through the torment my family and Cassie did. Darien Quote Link to comment Share on other sites More sharing options...
karrider Posted September 12, 2004 Report Share Posted September 12, 2004 Hi Darien, I think I know most of the lines that CL has been Proven in but would be interested in any more info you may be able to give, I don't think names can be named on a public list so perhaps you could send them privately??thanks karin Quote Link to comment Share on other sites More sharing options...
Darien Posted September 13, 2004 Report Share Posted September 13, 2004 Will do Karin, I had no intention of posting names here Give me a couple of days as I need to find them, as it's over 10 years now! Darien Quote Link to comment Share on other sites More sharing options...
karrider Posted September 13, 2004 Report Share Posted September 13, 2004 Thank you,I have found in the research and published details there is one dog that seems to be appearing a lot,it will be interesting to know if he appears in your pedigree too Karin Quote Link to comment Share on other sites More sharing options...
Darien Posted September 13, 2004 Report Share Posted September 13, 2004 I can guess the dog you are talking about. Where have you published the details? Also, if you are interested, or anyone else, I can give you the direct line of working BC's first introduced to Australisia. Darien Quote Link to comment Share on other sites More sharing options...
Darien Posted September 19, 2004 Report Share Posted September 19, 2004 Karin, I can not find my info, but then again I have been busy, but the BCCNSW have now published carriers of CL. Here is the site. http://www.bccnsw.com/ Darien Quote Link to comment Share on other sites More sharing options...
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