Storage Disorders of Sphingolipids

Sphingolipids are in a continuous state of turnover. They are catabolized by lysosomal enzymes by stepwise removal of sugar residues beginning at the nonreducing end 

Gjy[2 gangliosidosis, type II (Tay-Sachs variant Sandhoff s disease) 

Gaucher s disease (glucosyl ceramide lipidosis three types see text) 

Gjy[3 ganglioside accumulates most frequently other compounds occasionally. 

Same clinical picture as Tay-Sachs desease but progresses more rapidly no racial predilection hepatosplenomegaly, cardiomyopathy. 

---