Globoid cell leukodystrophy (galactocerebrosidosis)(GCL)
Also known as: Krabbe disease · Galactosylceramidase deficiency · GALC deficiency · Globoidzell-Leukodystrophie · Krabbe-Krankheit · …
Overview
What it is
Globoid cell leukodystrophy is an inherited metabolic disorder affecting the nervous system, caused by a deficiency in an essential enzyme. This deficiency leads to the buildup of harmful substances that destroy myelin, which is the protective sheath surrounding nerve fibers in the brain and spinal cord. Without this protective covering, the nerves cannot transmit signals properly, resulting in progressive neurological decline.
How it presents
Symptoms typically begin between 3 and 6 months of age, starting with tremors, muscle weakness, a stiff gait, and poor coordination. As the disease worsens, it progresses to paralysis, behavioral changes, and blindness.
Treatment
There is no cure for this fatal condition, and treatment is limited to supportive care and diagnostic confirmation. Managing the dog's comfort and confirming the diagnosis typically costs between 500 and 2000 EUR.
How it's tested
Labogen Komplettpaket (rassespezifisch)(Labogen-Panel)
Primary testDNA testRassespezifisches DNA-Panel (Labogen / Laboklin) — bündelt alle relevanten Erbkrankheiten der Rasse in einem Test.
Issuing body: Labogen
Result scheme: Clear/Carrier/Affected
Feragen Komplettpaket (rassespezifisch)(Feragen-Panel)
DNA testRassespezifisches DNA-Panel (Feragen, Österreich) — gleiches Konzept wie Labogen.
Issuing body: Feragen
Result scheme: Clear/Carrier/Affected
Embark Breed + Health Panel(Embark)
DNA testUS-Anbieter; 250+ genetische Erkrankungen + Abstammung. Anerkennung in DE/AT variiert je nach VDH-Verein.
Issuing body: Embark
Result scheme: Clear/Carrier/Affected
MyDogDNA Panel(MyDogDNA)
DNA testGenoscoper-basiertes Panel (Mars Petcare). 200+ Erkrankungen + Rassenkomposition.
Issuing body: MyDogDNA
Result scheme: Clear/Carrier/Affected
Affected breeds
15 of 15 breeds
Mandatory testLeonbergerMandated by Deutscher Club für Leonberger Hunde e.V.
RecommendedAustralian Kelpie
RecommendedAustralian Silky Terrier
RecommendedBeagle
RecommendedBluetick Coonhound
RecommendedCairn Terrier
RecommendedDalmatian
RecommendedEnglish Foxhound
RecommendedIrish Red Setter
RecommendedLabrador Retriever
RecommendedMiniature Poodle
RecommendedParson Russell Terrier
RecommendedSamoyed
RecommendedWest Highland White Terrier
RecommendedWorking Kelpie
Treatment cost
Estimated range of typical treatment cost. Actual cost depends on severity, clinic and region.
Frequently asked
What is Globoid cell leukodystrophy (galactocerebrosidosis)?
Globoid cell leukodystrophy is an inherited metabolic disorder affecting the nervous system, caused by a deficiency in an essential enzyme. This deficiency leads to the buildup of harmful substances that destroy myelin, which is the protective sheath surrounding nerve fibers in the brain and spinal cord. Without this protective covering, the nerves cannot transmit signals properly, resulting in progressive neurological decline.
How is Globoid cell leukodystrophy (galactocerebrosidosis) tested?
Tests currently in our database: Labogen Komplettpaket (rassespezifisch), Feragen Komplettpaket (rassespezifisch), Embark Breed + Health Panel, MyDogDNA Panel.
Which breeds are most affected?
Most commonly affected: Leonberger, Australian Kelpie, Australian Silky Terrier, Beagle, Bluetick Coonhound, Cairn Terrier, Dalmatian, English Foxhound.