Cerebellar Ataxia in Spinone

  MVS dogs or bitchs parent's have DNA markers indicating they are CLEAR of Cerebellar Ataxia.

                                                                       Great News !
The CA  AHT research in England has discovered the gene DIRECTLY related to CA (The AHT stands for the Animal Health Trust, who is involved with  the research.) 

Thank you!  Katherine Cooker

Cerebellar ataxia (CA) in the Italian Spinone is a serious neurological disease. In affected dogs the cerebellum, which is the part of the brain responsible for the co-ordination of motor movement in the body,  becomes diseased. Affected dogs are born normal, but clinical signs, which include the development of an unsteady gait, loss of co-ordination and poor balance usually appear in the first weeks or months of life. The disease is progressive; symptoms worsen during the first year of the  dogs life such that most dogs are put down before they are 1 year old. There is no treatment for the disease, which has an autosomal recessive mode of inheritance.
The genetic mutation that is responsible for the condition probably occurred spontaneously in a single dog but once in the population has been inherited from generation to generation like any other gene. 

The disorder shows an autosomal recessive mode of inheritance meaning, a dog has to inherit two copies of the defective gene (one from each parent) for it to be affected by the disease. Individuals with one copy of the defective gene and one copy of the normal gene are called carriers and show no clinical signs but can pass the defective gene onto their offspring. When two apparently healthy carriers are crossed, 25% (on average) of the offspring will be affected by the disease, 25% will be  clear and the remaining 50% will be carriers.
The region of the genome that contains the causal mutation has recently been identified at the Animal Health Trust. Using the information gained from this research, we have developed a linkage-based DNA test for the disease which we estimate will give an accurate result for between 95% and 98% of dogs tested. This test will detect those dogs which are carriers of the CA mutation, and those that are clear of the mutation.
Breeders will be sent results identifying their dog as belonging to one of three categories:

CLEAR: This DNA test detects markers close to the gene responsible for CA - our research has indicated that the test is 95-98% accurate. CLEAR dogs have 2 copies of the normal gene and will neither develop cerebellar ataxia, nor pass a cerebellar ataxia gene to their offspring.

CARRIER: This DNA test detects markers close to the gene responsible for CA - our research has indicated that the test is 95-98% accurate. 

CARRIER dogs have one copy of the normal gene and one copy of the mutant  gene that causes cerebellar ataxia. They will not develop cerebellar ataxia but will, if bred from, pass on the cerebellar ataxia gene to, on average, 50% of its offspring.

AFFECTED: This DNA test detects markers close to the gene responsible for CA - our research has indicated that the test is 95-98% accurate. AFFECTED dogs have 2 copies of the mutant gene that causes cerebellar ataxia and will develop the disease.

Carriers can still be bred to tested clear dogs. On average, 50% of such a litter will be clear and 50% carriers; there can be no affected produced from such a mating. Pups which will be used for breeding can themselves be DNA tested to determine whether they are clear or carrier.
    

Cerebellar Ataxia
Discovered in 1998, a hereditary condition called Cerebellar Ataxia (CA) was acknowledged in the Spinone Italiano, with the first suspect cases appearing in 1990. This disease cannot be treated, and of the puppies affected, none has survived past the age of 12 months. Cerebellar Ataxia is not "self-limiting."It is a very real problem that can only be resolved by never breeding from known CA lines.
It has been shown that the mode of inheritance of CA is a simple recessive gene that can be traced to a single dog, Trusco Dell Angelo Del Summano  Del Gaesten, imported to Great Britain from Italy. If both sire and dam carry the CA gene, affected puppies maybe born and will die. While death by this disease is heartbreaking. It is the surviving pups that present a threat to the Spinone Italiano as a breed. 50% of these puppies will be carriers of the lethal gene, and each pup from each of their litters  in turn will have a 50/50 chance of being a carrier. There was no genetic test for the CA gene, and no way of determining which of these descendants of Trusco were a carrier until it was bred to another Trusco descendant and CA affected puppies were produced.
The Spinone Club of America, the AKC parent club of the Spinone Italiano, were committed to the health of our dogs. We did not find ANY risk of perpetuating the CA gene, and thus risking producing CA affected puppies acceptable.

Spinone breeders must OFA certify Spinone for hip dysplasia prior to breeding, CERF eyes, and ensure that no Spinoni are bred from the known Cerebellar Ataxia carriers.
Self limiting, meaning the dogs that have CA die before they can be bred. The dogs that are affected by CA do die very young. Those dogs will have litter mates that are carriers of the gene while not being affected by it. The problem with the self-limiting concept is not the affected dogs, but the carrier dogs. Dogs that appear healthy but carry the CA gene. When a known carrier is bred to a clear line, fifty percent of the puppies will be carriers. We had no way of knowing which ones were clear and which ones were carriers. Those carrier puppies will never be affected, meaning they will never succumb to CA. However, they can pass the carrier genes to their own puppies. The more CA lines are bred, the more possible carriers we put into the gene/breeding pool. We've never had a KNOWN affected CA puppy in the United States, but there have been suspect deaths of puppies from affected lines. If we continue breeding CA carrier lines it is only a matter of time before these carriers will start to meet up. That's what the SCOA is afraid of. Once those carriers start meeting up, we will have CA affected puppies in the United States.

CA is not political, it is a disease and a real problem.All one has to do is look at other breeds to realize CA is a serious problem. There are many breeds that have serious genetic health problems that would not exist if certain dogs had not been bred. Boxers have a life threatening heart defect that can be traced back to a very popular stud dog, much like CA can be traced back to one dog. Unfortunately, this Boxer had been bred many times before anyone realized there was a problem. Now the breed is inundated with it. Doberman Pinschers also  have genetic heart problem. Twenty years ago breeders insisted that there was no proof it was genetic and continued to breed dogs whose parents had died of the disease. Now there are no lines of Dobies that are clear of the disease. Breeding CA lines is even worse than what happened with Boxers and Doberman Pinschers. We have a much smaller gene pool and we know the dogs that shouldn't be bred until a genetic test becomes available. 

Before you buy or breed a Spinone, it is important to know.

The problem is breeders who choose to pass on the carrier genes their dogs possess. Knowing what we know has happened in other breeds and knowing that some breeders continue to breed possible  carriers, is the problem real or political? The puppy does NOT only have a 2% chance of being a carrier.
Some CA line dogs currently being bred in the United States are from lines that have been cleared by pedigree research in Great Britain some have not. There is no way to know that any particular dog is a carrier without testing. 

Without testing no one knows which descendants of CA carriers are carriers and which are not until  they risk breeding dying puppies to find out. This is unnecessary and should be unacceptable to any breeder. We all love our dogs, but breeders who love the breed make responsible breeding choices. 

                                    MVS health screen their dogs for sound genetic quality. 

1. Our Goals are: Health, Good Temperament, Structure, and Field Savvy for a well rounded  dog. We hunt our Spinone but Spinone also make great family, obedience, tracking, agility, therapy, help, and much much more. Spinone are a wonderful combination, the best of both worlds.
2. It is important to insist on a minimum set of standards when acquiring any puppy.
   In its early history, the Italian Spinone was generally a healthy breed. However, indiscriminate breeding practices due to ignorance, lack of concern, and monetary gain changed the landscape dramatically. 
3. Our beloved breed has some issues that need to be addressed. These are hip dysplasia, elbow dysplasia, and thyroid conditions. Testing for eyes (CERF exam) C.A and DNA testing is also appreciated. Any dog that has had a history of unexplained seizures or any other obvious hereditary medical conditions must never be bred. Remember that these are serious health conditions that will severely impact the quality and length of life of any affected puppies. 
4. Health screening of breeding stock cannot guarantee that your puppy will be excluded from any of these problems but the risk factor is mitigated substantially.
5. A good breeder should be able to demonstrate testing of these issues by either providing a link to the Orthopedic Foundation for Animals records, CHIC records through AKC or they should be able to supply copies of the certified paperwork they received after the test screening was completed. If they do not or are not willing to provide this information, search out a breeder that is willing to disclose this information.
6. Your breeder should offer a minimum of 1-year written guarantee against genetic health conditions.

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