ME: I think I finally have these stupid storage disorders down

NBME: Not so fast, partner

Niemann-Pick is a lysosomal storage disease that is a deficiency in sphingomyelinase that leads to a buildup of sphingomyelin (Niemann picks his nose with his sphinger)

Sphingomyelinase is considered a lysosomal hydrolase

FA2020 pg 88--Lysosomal storage diseases

fts= AR, progressive neurodegeneration, hepatospenomegaly, cherry red macula, foam cells

deficienct enzyme= sphingomyelinase (a lysosmal hydrolase)--> build up of sphingomyelin

" No man picks his nose with his sphinger"

increased incidence in AShkenazi Jewish population

That's what I found: Sphingomyelinase is deficient in Niemann-Pick. Sphingomyelinase is an acidic lysosomal hydrolase. https://link.springer.com/chapter/10.1007/978-1-4613-1029-7_14