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
submitted by ∗corndog(18)
ME: I think I finally have these stupid storage disorders down
NBME: Not so fast, partner