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Lysosomal storage

Lysosomal storage disease

QuestionAnswerdescriptionsubstrate accumulated
fabry's alpha galactosidase a (peripheral neurophathy of hands/feet, angiokeratomas, cardiovascular/renal disease) ceramide trihexoside
goucher's b clucocerebrosidase ) (hsm, aseptic necrosis of femur, bone crisis, gaucher's cells glucocerebroside
niemannpick sphingomyelinase (progressive neurodegeneration, hsm, cherry red spot on macula sphingomyelin
tay sachs disease hexosaminidase a (progressive neurodegeneration, developmental delay, cherry red spot, lysozomes with onion skin) gm2 ganglioside
krabbe's bgalactosidase (peripheral neuropathy, dev't delay, optic atrophy) galactocerebroside
metachromatic leukodystrophy arylsulfatase a ( central and peripheral demyelination with ataxia, dementia cerebroside sulfate
hurler's alpha l iduronidase (dev't delay, gargoylism, airway obstrxn, corneal clouding, hsm) heparan sulfate, dermatan sulfate
hunter's iduronate sulfatase ) (mild hurler's + aggressive behavior, no corneal clouding heparan sulfate, dermatan sulfate
Created by: marcolhill