Hexosaminidase

Hexosaminidase is an enzyme involved in the hydrolysis of several molecules containing hexose.

Pathology
Deficiencies cause an inability to properly hydrolyze certain sphingolipids, causing these lipids to accumulate over time in lysosomes. Disorders of Hexosaminidase are responsible for the following conditions:


 * Tay-Sachs disease (type A only), lysosomes fill with GM2 gangliosides.
 * Sandhoff disease (types A and B), lysosomes cannot break down globosides.