What disease results from a deficiency in B-galactosidase?

The disease caused by a deficiency in B-galactosidase is GM1 gangliosidosis. This enzyme is crucial for the breakdown of certain glycolipids, particularly GM1 ganglioside. When B-galactosidase is deficient or absent, GM1 gangliosides accumulate, leading to neurological damage and other systemic issues.

In this context, it's important to clarify that while Krabbe's disease is associated with a deficiency in a different enzyme (galactocerebrosidase), it does not result from a deficiency in B-galactosidase. Each of the other diseases listed is associated with different enzyme deficiencies:

• Tay-Sachs disease is caused by a deficiency in hexosaminidase A, which leads to the accumulation of GM2 gangliosides.

• Niemann-Pick disease comes from a sphingomyelinase deficiency, causing sphingomyelin accumulation.

• Metachromatic leukodystrophy is linked to a deficiency in arylsulfatase A, leading to the accumulation of sulfatides.

Understanding these enzyme-specific diseases is essential for recognizing the metabolic pathways impacted by these deficiencies and the resulting clinical manifestations.