Mucopolysaccharidosis Type VII(MPS VII)
Also known as: Sly Syndrome · Beta-Glucuronidase Deficiency · GUSB deficiency · MPS 7 · Mucopolysaccharidose Typ VII
Overview
What it is
Mucopolysaccharidosis Type VII (MPS VII) is an inherited metabolic disorder belonging to a group called lysosomal storage diseases. It occurs when a deficiency in a specific enzyme prevents the body from breaking down complex sugar molecules, leading to a harmful buildup of waste products inside cells. This accumulation causes progressive damage to multiple organ systems, particularly the skeleton and connective tissues.
How it presents
Symptoms typically become noticeable in early puppyhood and progress rapidly. Owners may observe stunted growth (dwarfism), skeletal deformities, cloudy eyes, and severe difficulty moving.
Treatment
There is no cure for this condition, and treatment is limited to supportive care to manage pain and maintain comfort. This lifelong palliative care typically costs between 1,000 and 5,000 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
Mucopolysaccharidose Typ VII DNA-Test(MPS VII)
Primary testDNA testEin molekularbiologischer DNA-Test identifiziert die ursächliche Mutation im GUSB-Gen, um betroffene Hunde sowie symptomfreie Anlageträger (Träger) zuverlässig nachzuweisen.
Issuing body: other
Result scheme: Clear/Carrier/Affected
Mukopolysaccharidose Typ VII DNA-Test(MPS VII)
Primary testDNA testEin molekularbiologischer Gentest (PCR) zum Nachweis der ursächlichen Mutation im GUSB-Gen, die zu einem Mangel des Enzyms Beta-Glucuronidase führt.
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 Mucopolysaccharidosis Type VII?
Mucopolysaccharidosis Type VII (MPS VII) is an inherited metabolic disorder belonging to a group called lysosomal storage diseases. It occurs when a deficiency in a specific enzyme prevents the body from breaking down complex sugar molecules, leading to a harmful buildup of waste products inside cells. This accumulation causes progressive damage to multiple organ systems, particularly the skeleton and connective tissues.
How is Mucopolysaccharidosis Type VII tested?
Tests currently in our database: Labogen Komplettpaket (rassespezifisch), Mucopolysaccharidose Typ VII DNA-Test, Mukopolysaccharidose Typ VII DNA-Test, Feragen Komplettpaket (rassespezifisch), Embark Breed + Health Panel, MyDogDNA Panel.
Which breeds are most affected?
Most commonly affected: Brazilian Terrier, Canadian Shepherd.