Other Names for this Disease
- Beta galactosidase 1 deficiency
- GLB 1 deficiency
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GM1 gangliosidosis is a hereditary condition that is inherited in an autosomal recessive manner. This means that to be affected, a person must have a mutation in both copies of the responsible gene in each cell. Affected people inherit one mutated copy of the gene from each parent, who is referred to as a carrier. Carriers of an autosomal recessive condition typically do not have any signs or symptoms (they are unaffected). When 2 carriers of an autosomal recessive condition have children, each child has:
- a 25% (1 in 4) chance to be affected
- a 50% (1 in 2) chance to be an unaffected carrier like each parent
- a 25% chance to be unaffected and not be a carrier
GM1 gangliosidosis is type-specific within families. This means that people with a family history of the condition are generally only at increased risk for the specific type of GM1 gangliosidosis in the family.
Last updated: 11/17/2015
- Tegay D. GM1 Gangliosidosis. eMedicine. March 29, 2012; http://emedicine.medscape.com/article/951637-overview. Accessed 4/3/2012.
- About Gangliosidosis-1 (GM-1). National Tay-Sachs and Allied Diseases Association, Inc.. http://www.ntsad.org/index.php/the-diseases/gm-1. Accessed 4/3/2012.