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A DNA test for Globoid Cell Leukodystrophy (GCL) in dogs
What is GCL?
GCL, also called Krabbe's disease, is a degenerative disease of the white
matter of the brain and spinal cord. Segmental degeneration of some peripheral
nerves may also occur. It is the result of a genetic deficiency of an enzyme
(galactocerebroside b-galactosidase) that is involved in the breakdown of certain
lipids (fats) in the brain and spinal cord.
Clinical signs begin early in life and progress rapidly. GCL may occur as
early as four weeks of age and nearly always occurs before six months of age.
Lack of coordination, weakness, stumbling, loss of control of the hindquarters,
and tail tremors may be observed. Then the dog will develop a wide stance,
incoordination, and posterior or total limb paralysis. The dog may become blind
or fail to recognize familiar individuals. The response to painful stimuli is
generally unaffected.
GCL is inherited as an autosomal recessive trait. Diagnosis can be
made by measuring the mean galactocerebroside b-galactosidase enzyme activity in
white blood cells. Heterozygotes can be identified, since affected dogs have
about 15-18% and carriers about 50% of the mean activity found in the cells
of normal dogs.
A DNA test for GCL
Breeds at risk
Breeding policy
Testing for GCL
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