NCL of Neuronal Ceroid Lipofuscinosis
Background
NLC is a serious neurologic disease, causing the degeneration of the central nervous system. The illness is caused by incorrect storage of energy in sections of cells (lysosomen). The symptons arisee mostly between 3 to 5 years and cause uncontrolled movements, loss of conciousness and abnormal behaviour. The disease develops progressively, which means that complaints will become more frequent and more serious. In the end the dog will no longer be able to walk.
Age
The illness begins at different ages and it is difficult to judge at what age the first symptons will appear. There can be differences between siblings and breeds.
Characteristic of the illness
This disorder causes loss of brain functioning
Examination
A DNA test is availablle from a number of labs to see whether a dog has this illness.
Our club uses HTTPS://www.animaldnadiagnostics.co.uk. The test is done by the vet and sent to the lab.
Inheritance
These characteristics are inherited in a autosomal and recessive manner; This means that an animal can be free from (homozygote normal), sufferer (homozygote malfuntioning) or carrier (heterozygote).
Free, carrier, affected
An animal is free and has two healthy alleles. When used for breeding this animal will not have any disorder nor transmit it to the next generation
Free
The dog has two healthy alleles. This dog when used for breeding will not develop any disorder and cannot transmit to the next generation.
Carrier
The dog has one healthy and one mulfunctioning. The animal will transmit the mutant (malfuncti oning) allele to half his descendants. Carriers can sometimes also be affected by the malfuntioning allele, but will generally have no symptons
Affected
The dog has two malfuntioning alleles. Sufferers will transmit the malfunctioning allele to all their descendants and will have symptons of the illness
The club demands that their breeders have their breeding animals DNA tested, if both parents are free from you can skip one generation and the test is not necessary; the second generation however needs to be tested again.
| Following combinations are allowed |
- clear X clear
- carrier X clear
- carrier x carrier is not allowed !!
- Breeding with a affected dog is not allowed!!
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| Thanks to this DNA test we can breed more conciously and avoid combinations that are not allowed. |
Bron: VHLGenetics |
