Lipid metabolism mnemonic

Sphingolipidoses with their enzyme deficiency and inheritance

The mnemonic

Fabry is the odd one out: X-linked

What each word stands for

  • FabryAlpha-galactosidase A deficiency, X-linked recessive
  • GaucherGlucocerebrosidase deficiency, autosomal recessive
  • Niemann-PickSphingomyelinase deficiency, autosomal recessive
  • Tay-SachsHexosaminidase A deficiency, autosomal recessive

Why it matters clinically

Most lysosomal storage diseases are autosomal recessive; Fabry and Hunter are X-linked exceptions.

Where it stops being reliable

Hunter syndrome (a mucopolysaccharidosis) is also X-linked and is not a sphingolipidosis.

Teaching aid, still awaiting independent review. Confirm facts in a standard source.

More mnemonics

Common questions

What is the Lipid metabolism mnemonic?

Fabry is the odd one out: X-linked. It helps you recall: Sphingolipidoses with their enzyme deficiency and inheritance.

What does "Fabry" stand for?

Alpha-galactosidase A deficiency, X-linked recessive

What does "Gaucher" stand for?

Glucocerebrosidase deficiency, autosomal recessive

What does "Niemann-Pick" stand for?

Sphingomyelinase deficiency, autosomal recessive

What are all the parts of the Fabry is the odd one out: X-linked mnemonic?

Fabry: Alpha-galactosidase A deficiency, X-linked recessive; Gaucher: Glucocerebrosidase deficiency, autosomal recessive; Niemann-Pick: Sphingomyelinase deficiency, autosomal recessive; Tay-Sachs: Hexosaminidase A deficiency, autosomal recessive.

Which topic does this mnemonic belong to?

It belongs to Lipid metabolism in Biochemistry.

Where does this mnemonic stop being reliable?

Hunter syndrome (a mucopolysaccharidosis) is also X-linked and is not a sphingolipidosis.

How is this useful clinically?

Most lysosomal storage diseases are autosomal recessive; Fabry and Hunter are X-linked exceptions.

How do I make this mnemonic stick?

Say it aloud, write it from memory tomorrow, then again in a week. Then test yourself on a Biochemistry question about Lipid metabolism.

Is this a standard mnemonic?

It is a teaching aid written or adapted by our team. Some resemble ones students share informally. Check any fact that matters in a standard source.