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- lacks heXosaminidase A
- build up of GM2
- Cherry-RED macula
- lacks α-Galactosidasebuild up of Globosides
- only X-linked disorder; pain in extremeties with reddish-purple skin rashes
- lacks glucocerebrosidasebuild up of Glucocerebroside
- Hepatosplenomegaly; "wrinkled paper inclusions"
- lacks Sphyngomyelinase
- build up of Sphyngomyelin
- "Zebra bodies"; "Foamy" macrophages
"No man picks his nose with his sphinger
- lack of B-galactosidase
- build up of Galactocerebroside
- muslce weakness; breakdown of myelin sheath