GM1 Gangliosidosis(GM1)
Also known as: GM1-Gangliosidose · Acid beta-galactosidase deficiency · GLB1 deficiency · Lysosomale Speicherkrankheit GM1 · Lysosomale Speicherkrankheit · …
Overview
What it is
GM1 gangliosidosis is an inherited lysosomal storage disease that primarily affects the nervous system. It occurs when a deficiency in a specific enzyme, beta-galactosidase, prevents the body from breaking down certain fatty sugar compounds. Over time, these compounds build up to toxic levels inside cells, causing progressive damage to the brain and spinal cord.
How it presents
Symptoms typically appear in puppies between 4 and 6 months of age, starting with tremors, muscle weakness, and difficulty coordinating movements. The condition progresses rapidly, eventually leading to blindness and seizures.
Treatment
This disease is currently incurable and unfortunately fatal. Veterinary care is focused entirely on keeping the dog comfortable, with palliative care and humane euthanasia costs typically ranging from 500 to 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
Gangliosidose (GM1/GM2) DNA-Test(GM1/GM2)
Primary testDNA testEin molekularbiologischer DNA-Test zum Nachweis der ursächlichen Mutationen für GM1- oder GM2-Gangliosidose im Erbgut des Hundes.
Issuing body: Labogen
Result scheme: Clear/Carrier/Affected
GM1-Gangliosidose DNA-Test(GM1)
Primary testDNA testEin molekularbiologischer DNA-Test zum Nachweis der Mutation im GLB1-Gen, die für den Mangel an Beta-Galaktosidase verantwortlich ist.
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
Treatment cost
Estimated range of typical treatment cost. Actual cost depends on severity, clinic and region.
Frequently asked
What is GM1 Gangliosidosis?
GM1 gangliosidosis is an inherited lysosomal storage disease that primarily affects the nervous system. It occurs when a deficiency in a specific enzyme, beta-galactosidase, prevents the body from breaking down certain fatty sugar compounds. Over time, these compounds build up to toxic levels inside cells, causing progressive damage to the brain and spinal cord.
How is GM1 Gangliosidosis tested?
Tests currently in our database: Labogen Komplettpaket (rassespezifisch), Gangliosidose (GM1/GM2) DNA-Test, GM1-Gangliosidose DNA-Test, Feragen Komplettpaket (rassespezifisch), Embark Breed + Health Panel, MyDogDNA Panel.
Which breeds are most affected?
Most commonly affected: Alaskan Husky, Shiba Inu, Shikoku.