Question:
Fish odor syndrome is caused by deficiency of which enzyme?
Fish odor syndrome is caused by deficiency of which enzyme?
Updated On: Jul 12, 2025
- Fumarylacetoacetate hydrolase
- Methane monooxygenase
- Monooxygenase 3 (FMO3)
- D-amino acid oxidase
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The Correct Option is C
Solution and Explanation
Fish odor syndrome, also known as Trimethylaminuria, is a metabolic disorder caused by a deficiency of the enzyme Monooxygenase 3 (FMO3). This enzyme is responsible for the oxidation of trimethylamine (TMA), a compound with a strong fishy odor, into trimethylamine N-oxide (TMAO), which is odorless. When FMO3 is deficient, TMA accumulates in the body and is excreted in sweat, urine, and breath, leading to the characteristic odor associated with the syndrome.
| Enzyme | Function |
|---|---|
| Fumarylacetoacetate hydrolase | Involved in tyrosine metabolism |
| Methane monooxygenase | Oxidizes methane to methanol |
| Monooxygenase 3 (FMO3) | Oxidizes trimethylamine to trimethylamine N-oxide |
| D-amino acid oxidase | Degrades D-amino acids |
In summary, the deficiency of Monooxygenase 3 (FMO3) leads to the symptoms of Fish odor syndrome due to its role in processing trimethylamine.
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