Feb 7 2014

What is Trimethylaminuria (TMAU) or the fish-odour syndrome? Trimethylamine (TMAU) is a byproduct formed as a result of the body's conversion of the nutrient 'choline' into essential compounds for use by the nervous system and other body systems.
This byproduct, composed of methyl and amines, is a highly odourous chemical but under normal functioning the body deodorizes this chemical prior to elimination. A gene releases an enzyme called flavin mono-oxidase which is responsible for making the byproduct odourless. This enzyme adds oxygen to the chemical, converting it to Trimethyl-N-oxide and thereby neutralising the odour. The chemical is excreted through all pores in the body, including the skin, mouth and nose. In some individuals the body fails to oxidize this chemical, giving the person a very unpleasant odour. This condition is called Trimethylaminuria or TMAU.

Rev Neurol (Paris). 2011. L-carnitine treatment and fish odor syndrome: an unwaited adverse effect. Levocarnitine treatment is usually well tolerated, with essentially dose-dependent diarrhea as the main induced adverse effect. We report a case of fish odor syndrome during levocarnitine treatment which resolved after levocarnitine discontinuation. This adverse effect seems to be correlated with excedent carnitine intake and might be expressed when the elimination pathway becomes saturated or in a situation of deficiency enzymatic metabolism.

Q. I have a rare disorder called TMAU and would like to know what kind of enzymes or vitamin supplements you can recommend based on the description of the illness below. NOTE: My body`s enemy is anything with choline in it (pretty much most protein). Any suggestions would be greatly appreciated.   
   A. We are not experts on this topic so we don't know.