What is the enzyme deficiency associated with Krabbe disease?

Krabbe disease is associated with a deficiency of the enzyme galactocerebrosidase. This enzyme is critical for the metabolism of galactolipids, specifically in the breakdown of galactocerebroside into galactose and ceramide. When there is a deficiency in galactocerebrosidase, it leads to an accumulation of toxic metabolites, which primarily affects the myelin sheath of nerve cells in the central nervous system. This accumulation causes severe neurological symptoms and progressive deterioration seen in individuals with Krabbe disease.

The other options refer to different enzyme deficiencies associated with other lysosomal storage diseases. Sphingomyelinase is involved in Niemann-Pick disease, while hexosaminidase is related to Tay-Sachs disease. Galactosylceramidase is responsible for the biosynthesis and metabolism of galactolipids but does not directly relate to Krabbe disease. Understanding these distinctions is important for recognizing the specific metabolic pathways and associated conditions.