Mucopolysaccharidosis IIIB(MPS IIIB)
Also known as: Mucopolysaccharidosis Type IIIB · Sanfilippo Syndrome B · Sanfilippo Type B · NAGLU deficiency
Overview
What it is
Mucopolysaccharidosis IIIB is an inherited lysosomal storage disease that primarily affects the brain and nervous system. It is caused by a deficiency in a specific enzyme needed to break down complex sugar molecules. Without this enzyme, these sugars accumulate inside the body's cells, leading to progressive cellular damage.
How it presents
Symptoms typically become noticeable between 2 and 4 years of age. Owners may observe progressive neurological issues, such as tremors, a loss of balance, coordination difficulties, and changes in the dog's temperament.
Treatment
There is no cure for this condition, and treatment is limited to supportive care to keep the dog comfortable. Managing these symptoms throughout the dog's life typically costs between 1,000 and 3,500 EUR.
How it's tested
Mucopolysaccharidose Typ IIIB (MPS IIIB) DNA-Test(MPS IIIB)
Primary testDNA testDer DNA-Test identifiziert die ursächliche Mutation im NAGLU-Gen, die für den Mangel des Enzyms Alpha-N-Acetylglucosaminidase verantwortlich ist.
Issuing body: Labogen
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 IIIB?
Mucopolysaccharidosis IIIB is an inherited lysosomal storage disease that primarily affects the brain and nervous system. It is caused by a deficiency in a specific enzyme needed to break down complex sugar molecules. Without this enzyme, these sugars accumulate inside the body's cells, leading to progressive cellular damage.
How is Mucopolysaccharidosis IIIB tested?
Tests currently in our database: Mucopolysaccharidose Typ IIIB (MPS IIIB) DNA-Test.
Which breeds are most affected?
This condition isn't tied to a specific breed in our database.