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Neuronal Ceroid-Lipofuscinoses (NCL8)

What is Neuronal Ceroid-Lipofuscinoses (NCL8)?                                                                

NCL is a disease that is passed from parents to their offspring. It causes  gradual degeneration of the nervous system.  ​

This degeneration occurs when waste accumulates and causes the nerve cell to die. The death of these cells in the nervous system is what causes adult Saluki’s  to develop severe neurologic signs. Most dogs begin to develop signs around 1-1.5 years old.

 

NCL8 is a rare but serious disease, and testing of all dogs is strongly encouraged

What Are The Clinical Signs and Symptoms of NCL8?                                                         

Affected dogs show progressive neurological signs, meaning that as time goes on the signs and symptoms get worse. Neurologic signs can include: - Dragging the feet - Lack of coordination, and stumbling (ataxia) - Swimming movements  - Inability to stand - Difficulty chewing and eating - A head tilt, or drooping ears or lips - Loss of vision and blindness - Anxiety - Seizures - Loss of ability to hold urine and stool (incontinence)

What Is The Treatment for Saluki's Who Have NCL8?                                                        

Unfortunately at this time there is no treatment for this disease. Animals who have been  diagnosed with NCL are euthanized as their quality of life declined soon after diagnosis.

 

Therefore genetic testing of all Saluki is the best way to prevent animals from passing on NCL. 

Genetic Testing For NCL8                                                                                                            

A genetic test should be performed on all Salukis, ideally at 4-6 months old (but it can be done earlier).  Testing can be performed with one of the two labs below. 

Embark

Choose the Embark “Purebred- Breed+Health Test Kit” - Embark will send a test kit to your home, with instructions on how to collect samples, and a pre-paid shipping container.  - You are responsible for communicating results to the OFA (directions can be found in the OFA section of this website)

Wisdom Panel

Choose the “Wisdom Panel Premium Test Kit” - Wisdom Panel will send a test kit to your home, with instructions on how to collect samples, and a pre-paid shipping container.   - You are responsible for communicating results to the OFA (directions can be found in the OFA section of this website)

Possible Test Results and Interpreting Them                                                                        

Every animal has TWO copies of most genes- one from the mother and one from the father. For a Saluki to have signs and symptoms of NCL they must have TWO copies of the NCL gene.  

 

CL- Affected- Is the code for the NCL gene 

N- Normal ​

There are three possible test results: 1. N/N:  TWO Normal Genes- No Copies of the NCL Gene  Dogs with N/N do NOT have any copies of the NCL gene and will not develop signs or pass it to their offspring.  2. CL/CL:  TWO copies of the NCL Gene Dogs with CL/CL genotype have TWO copies of NCL variant found and are expected to develop the disease. 3. N/CL: ONE normal gene and ONE copy of the NCL gene. If a Saluki has ONE copy of the gene they will not become sick. These dogs are called carriers. It is possible for them to pass this to their offspring. - If two carriers are mated, approximately 25% of the puppies are predicted to develop the disease and 50% are predicted to be carriers.  - It is NEVER recommended to breed two carriers. And discouraged to breed a carrier to a non-carrier.

Resources and References                                                                                                                          

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