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A DNA test for Globoid Cell Leukodystrophy (GCL) in dogs



What is GCL?

A DNA test for GCL

GCL is inherited as an autosomal recessive trait that was found in West Highland White Terriers and in Cairn Terriers. The University of Pennsylvania, School of Veterinary Medicine, discovered the molecular defect causing the disorder and developed DNA tests to detect affected animals and carriers.
Breeders should consider screening their breeding stock for the presence of carriers of GCL.
Applying the DNA test allows breeders to classify their potential breeding animals at a young age as genetically healthy ("clear"), "carrier" or "affected" in order to select effectively against the genetic disorder.

The GCL DNA test yields three possible outcomes:
  • Your dog is clear (and has two healthy alleles: GCL/GCL), it will not suffer from GCL and, just as important, it will not pass on the defect (the defective allele) to the next generation.
  • Your dog is a carrier (and has one healthy allele and one defective one: GCL/gcl), it will not suffer from GCL but it will pass on the defective allele to half of its offspring.
  • Your dog is affected (and has two defective alleles: gcl/gcl), it will most probably die at young age because of an ongoing storage of lipids in its cells. So it is one of the animals that will suffer from GCL.


Breeds at risk

Breeding policy

Testing for GCL