Beta-mannosidosis(BM)
Also known as: Beta-Mannosidose · Lysosomal storage disease beta-mannosidosis · β-mannosidosis · B-mannosidosis
Overview
What it is
Beta-mannosidosis is a severe, inherited metabolic disorder where a deficiency in the enzyme beta-mannosidase prevents the body from breaking down complex sugar molecules. These sugars build up inside cells, particularly damaging the central nervous system, which includes the brain and spinal cord. This cellular accumulation leads to progressive and irreversible neurological damage.
How it presents
Symptoms typically appear within the first few weeks of life. Owners will notice severe neurological signs, including uncontrollable body tremors, a lack of coordination, deafness, and profound weakness.
Treatment
There is no cure for this fatal condition, and veterinary care is limited to supportive care and humane euthanasia. The associated costs for diagnosis and end-of-life care typically range from 250 to 1000 EUR.
How it's tested
Beta-Mannosidose DNA-Test(BM)
Primary testDNA testEin molekularbiologischer DNA-Test zum Nachweis der verantwortlichen Mutation im MANBA-Gen, die zur Beta-Mannosidose führt.
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 Beta-mannosidosis?
Beta-mannosidosis is a severe, inherited metabolic disorder where a deficiency in the enzyme beta-mannosidase prevents the body from breaking down complex sugar molecules. These sugars build up inside cells, particularly damaging the central nervous system, which includes the brain and spinal cord. This cellular accumulation leads to progressive and irreversible neurological damage.
How is Beta-mannosidosis tested?
Tests currently in our database: Beta-Mannosidose DNA-Test.
Which breeds are most affected?
This condition isn't tied to a specific breed in our database.