Lysosomal stroge disease mneumonics

1. 1. fabry disease
2. gaucher disease-glucocerebrosidase
3. neimann-pick-sphingomyelinase
4. tay-sachs- hexominidase A
5. krabbe
6. metochromatic dystrophy
7. hurler syndrome
8. hunter disease

A Fabulous Alpha-Male that breaks the hearts of 3 fragile women by peeing on them” 

Fabry’s Disease results in cardiovascular (hearts) and renal (peeing) disease, due to a lack of (alpha male), alpha-galactosidase A and accumulation of Ceramide Trihexoside (Three fragile women)

“It’s gauche to glue things into your bones with tissue paper.”
Gaucher’s Disease causes hepatosplenomegaly and aseptic necrosis of femur, bone crises (bones) due to deficiency of Glucocerebrosidase.
I’m also partial to the following mnemonic: “Glucose is so gauche. Carrying a big liver around? That’s really tacky, just get a purse. Go hide and cry into some crumpled tissues.”
Gauche for Gaucher’s, hepatosplenomegaly, macrophages look like crumpled tissue paper, glucocerebroside accumulates.

“I hope No man picks his bloody foaming nose with his sphinger” (Courtesy of First Aid 2013)
Niemann-Pick Disease causing progressive neurodegeneration and hepatosplenomegaly, with both a cherry-red spot on macula (bloody) and foam cells (foaming nose), due to a lack of Sphingomyelinase (Sphinger), causing accumnulated sphingomyelin.
The ‘I Hope’ prefix is to indicate hepatosplenomegaly, which differentiates from the other cherry-red spot in macula disease, Tay-Sachs.

“I put my sachs of onions and cherries in the back of my GM truck. I sure hope they don’t get hexed.”
Tay Sachs – Lysosomes with onion skin, hexosaminidase A is deficient, GM2 gangloside is what accumulates, and of course – cherry red spot.

“I’m Krabbe because I can’t see the globes in the Galaxy.”
Krabbe’s Disease causes optic atrophy (can’t see), globoid cells and galaxy for galacto

“Aryl had many colorful and cerebral brothers, they made him clumsy and crazy.” 
Metachromatic Dystrophy – Arylsulfatase A is messed up, cerebroside sulfate accumulates, it has ataxia and dementia. Colorful is for metachromatic.

“Hurlers ran around in a daze, they couldn’t see or breathe”. “When you run hard hard you hurl, you’re bent over and can’t breathe, and you take off your lid to see.”
Hurler’s Syndrome – Results in developmental delay, gargolyism (bent over), airway obstruction (can’t breathe), corneal clouding (can’t see) and hepatosplenomegaly. Results to a deficiency in a-L-iduronase, accumulating heparan and dermatan sulfate.

“They are aggressive hunters who see clearly and hit the x with their arrows.” 
Hunter’s Disease manifests with aggressive behavior, no corneal clouding, and is X-linked Recessive inherited. It’s due to a lack of iduronate sulfase, and an accumulation of heparan and dermatan sulfate. 

Gaucher disease:

Tay-sachs:

Niemann pick :

Fabry disease: