Sulfur Metabolism

One mother has commented that her child smelled like green onions and apparently certain scents have been reported by other PWS parents. Volatile sulfur-containing compounds are known to have scents described as rubbery, skunky, or like onion, garlic, cabbage, kerosene, etc. This suggests the diffusion from skin of a sulfur-containing substance, which in turn suggests a problem with the metabolism of sulfur, including perhaps one or more sulfur-containing proteins.

• The smell of dimethyl sulfide is reminiscent of asparagus, corn, or molasses, while ethanethiol is more onion or rubber-like. n-butyl mercaptan is responsible for the skunk smell, while hydrogen sulfide is generally described as rotten-egg-like.
• Sulfur-containing proteins in the body:
  • methionine
  • cysteine
  • glutathione
• Pantothenate and/or pyridoxine (vitamin B6) deficiency in some wine yeasts impair sulfur metabolism.