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A DNA test for Globoid Cell Leukodystrophy (GCL) in dogs
What is GCL?
A DNA test for GCL
Breeds at risk
The available DNA test for GCL has been proven to detect carriers and affected
animals in West Highland White Terriers and Cairn Terriers. However the
disorder is not unique to those breeds. In literature Krabbe's disease has been reported
in several other breeds as well. For the time being we must assume that GCL in other
breeds is caused by a different mutant allele, maybe even on another locus. GCL
occurs on a sporadic basis in Dalmatians, Irish Setters, Miniature Poodles,
Beagles, Blue Tick Coonhounds, Basset Hounds and Pomeranians.
In future more breeds will be added to that list.
In the past, when breeds were developed, often breeding stock of other breeds was used to
improve certain characteristics. In the process of introducing the desired qualities (genes)
into a breed, it cannot be avoided that many neutral as well as undesirable genes cross the
barrier between breeds.
For the time being, in cases when symptoms of a neuropathologic storage disease, or
even of GCL, are found in a breed with "family ties" with West Highland White Terriers
or Cairn Terriers, checking with the available DNA test could be a "best guess". If
it can be proven that the defective alleles are present, applying that DNA test will help
the breeders in their selection against the disorder. From there on it is one out of two,
solving an existing breed problem or preventing a future breed problem from developing.
Breeding policy
Testing for GCL
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