Problem
Gangliosidosis
Other Names:
Disorders of ganglioside metabolism Nature:
Gangliosidosis contains different types of lipid storage disorders caused by the accumulation of lipids known as gangliosides. There are two distinct genetic causes of the disease. Both are autosomal recessive and affect males and females equally.
Background:
There are two forms of gangliodosis. GM1 gangliodsidosis affects the enzyme á-galactosidase and produces storage of GM1 ganliosides; the affected chromosome is number 3. GM2 gangliosidosis, AB variant affects the production of the GM2 activator protein enzyme and causes the storage of GM2 ganglioside; the affected chromosome is 5. Tay-Sachs disease is often called GM2 gangliosidosis, Type I; Sandhoff disease GM2 gangliosidosis Type 2.
Broader Problems:
Congenital disorders of lipid metabolism Related Problems:
Sandhoff diseaseTay-Sachs disease Subject(s):
Biosciences → BiochemistryMedicine → Pathology
Problem Type:
G: Very specific problems Date of last update
22.06.2016 – 04:28 CEST