Benders'dictionary of nutrition and food technology

297maple syrup urine disease A rare genetic disease affectingcatabolism of the branched-chain amino acids leucine, isoleucineand valine, due to deficiency of branched-chain ketoaciddehydrogenase (EC 1.2.4.4), leading to accumulation of highconcentrations of these amino acids and their keto-acids inplasma and urine. The keto-acids give the urine a characteristicsmell like that of maple syrup. If untreated, leads to severemental retardation and death in infancy.marasmic kwashiorkor The most severe form of protein–energymalnutrition in children, with weight for height less than60% of that expected and the oedema and other signs ofkwashiorkor.marasmus See protein–energy malnutrition.marc (1) French; spirit distilled from the fermented residue ofgrape skins, stalks and seeds after the grapes have been pressedfor wine making. The same as grappa (Italian), bagaciera(Portugal) and aguardiente (Spain). Often a harsh raw spirit,drunk young, although some are matured and smooth.(2) Insoluble residue after extraction of soluble material fromsugar beet; mainly non-starch polysaccharides, used as livestockfeed.margarine (butterine, lardine, oleomargarine) Emulsion of about80% vegetable, animal and/or marine fats and 20% water, originallymade as a substitute for butter. Usually contains emulsifiers,antispattering agents, colours, vitamins A and D (sometimesE) and preservatives.Ordinary margarines contain roughly equal proportions ofsaturated, mono-unsaturated and polyunsaturated fatty acids;special soft varieties are rich in polyunsaturated fatty acids. Lowfatspreads are made with 10–60% fat and correspondinglyhigher contents of air and water and less energy, and generallycannot legally be called margarine.Kosher (and vegetarian) margarine is made only fromvegetable oils, because ordinary margarine may include animalfats. It is fortified with carotene (which is derived from vegetablesources) as the source of vitamin A, instead of retinol (which maybe obtained from non-kosher sources).mariculture aquaculture in saline environments.marigold Pot or common marigold (Calendula officinalis); petalsare used as flavouring and colouring, sometimes as a substitutefor saffron.marinade Mixture of oil with wine, lemon juice or vinegarand herbs in which meat or fish is soaked before cooking,both to give flavour and to make it more tender. Hence tomarinate.