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NBME 19 Answers

nbme19/Block 1/Question#26 (reveal difficulty score)
Niemann-Pick disease is characterized by ...
Decreased lysosomal hydrolase activity🔍,📺
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 +8 
submitted by corndog(15),
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EM: I knith I lnalyfi ahve eehst dustip oatersg dsoedrrsi nwod

:MNEB Nto so tsaf, ratpner

my_second_membership  ^ voted “best username” in high school +2  
i_hate_it_here  Same bro +  
chaosawaits  ME: OH DAMN! I ALMOST FELL FOR THE TRICK! I'M FINALLY WINNING! ALL THIS HARD WORK AND SACRIFICE DOES HAVE MEANING! NBME: lololololol +  



 +6 
submitted by cassdawg(1446),
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kNnamcn-Peii is a loaslmyso oertgas seeisda atht is a dyeciiefcn ni sepghnemosnaiily tath ldesa ot a bduupli of olpmihsyiegnn mnn(iaeN kscip ish oesn iwth ish ghp)nsrie

mlinSipeanyghsoe is cedrseoind a ololysmsa elrahsdyo

chaosawaits  I'm guessing the reason "decreased synthesis of ceramide" isn't the answer then is based on FA2019 p88. Since there are 3 different paths to make ceramide, losing one throught Niemann-Pick disease will not significantly decrease the ceramide production +  



 +0 
submitted by icedcoffeeislyfe(49),
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00A2F2 gp Lol8-smasy8-o orsgtae dsssaiee

=sft AR, epvorgisres gte,nnreneaueioord yoealpemho,eagsntp cyerrh edr uaclam, amfo clsel

eftiedcnci eenzmy= iyeamopnlhgnssei (a olaysslm )or;sde&a-hglyt- udlib up fo oenimshliynpg

" oN amn pcski his nose wthi ihs n"pghirse

sriencead edcnicien in SaineAkhz eiwJsh uioonltpap

chaosawaits  Niemann definitely does lol that little nose-picker +  



 +0 
submitted by mdk(1),

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