A Nonsense Mutation in the FMO3 Gene Underlies Fishy Off-Flavor in Cow's Milk

Fish-odor syndrome or Trimethylaminuria (OMIM #602079) in humans is an inborn error of metabolism associated with a characteristic fishy body odor due to elevated levels of trimethylamine (TMA) in body fluids. It is caused by loss-of-function mutations in FMO3 encoding flavin-containing mono-oxygenase 3. A fishy off-flavor is occasionally observed in cow's milk and it has been established recently that this phenotype is due to elevated TMA levels. Here, we report that fishy off-flavor in cow's milk is caused by a nonsense mutation (R238X) in the bovine FMO3 ortholog. RT–PCR analysis indicated that the mutant transcript is present in a very low amount. The mutation was found to be surprisingly common (q = 0.155) in one breed of cattle. [The sequence data described in this paper have been submitted to GenBank with accession nos. AF488417–AF488422. The following individuals kindly provided reagents, samples, or unpublished information as indicated in the paper: K. Sandberg and I. Hansson.

Composition of poorly and non-coagulating bovine milk and effect of calcium addition

v2010o

Report of the NORMAN Workshop on Emerging Environmental Pollutants: Key Issues and Challenges Stresa, Italy 19-20 June 2006

Since the 1970s the impact of chemical pollution has focused almost exclusively on lists of conventional priority pollutants, especially those referred to as PBT (fulfilling criteria of Persistance, Bioaccumulation and Toxicity) or POPs (Persistent Organic Pollutants). The number of regulated, conventional pollutants, however, represents only a small part of the total number of potential pollutants. Emerging pollutants pose increasing threats to public health and ecosystems. Emerging pollutants are not necessarily new chemicals, and some of them have often long been present in the environment, but their presence and significance are only now being elucidated. How are emerging pollutants identified? How do emerging pollutants become regulated pollutants? What investigations should be undertaken when an emerging pollutant is identified? What are the substances that are being identified as emerging pollutants today, and what information is currently missing or required in terms of monitoring and assessment of their associated risks? Information on these substances is often difficult to obtain and the methods used are often at a research and development stage, or not appropriate for harmonisation at the European level. This makes comparison and interpretation of data very difficult, and represents a major difficulty for decision-making by the authorities. To discuss these issues from a wide perspective a workshop on ‘Emerging environmental pollutants: key issues and challenges’ was held in Stresa, Italy on 19-20 June 2006 as part of the activities of the EU-funded NORMAN project (Network of reference laboratories, research centres and related organisations for monitoring of emerging environmental pollutants) . The workshop was organised with the collaboration of: INERIS - Institut National de l’Environnement Industriel et des Risques (France) JRC-IES – Joint Research Centre – Institute for Environment and Sustainability (EC) EA – Environment Agency for England and Wales (UK).JRC.H.5-Rural, water and ecosystem resource