Maple Syrup Urine Disease supplements
Maple Syrup Urine Disease
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Thiamin (also spelled "thiamine") is a water-soluble B-complex vitamin, previously known as vitamin B1 or aneurine. Thiamin was isolated and characterized in the 1920s, and thus was one of the first organic compounds to be recognized as a vitamin. Thiamin is involved in numerous body functions, including: nervous system and muscle functioning; flow of electrolytes in and out of nerve and muscle cells (through ion channels); multiple enzyme processes (via the coenzyme thiamin pyrophosphate); carbohydrate metabolism; and production of hydrochloric acid (which is necessary for proper digestion). Because there is very little thiamin stored in the body, depletion can occur as quickly as within 14 days. Severe chronic thiamin deficiency (beriberi) can result in potentially serious complications involving the nervous system/brain, muscles, heart, and gastrointestinal system. Dietary sources of thiamin include beef, brewer's yeast, legumes (beans, lentils), milk, nuts, oats, oranges, pork, rice, seeds, wheat, whole grain cereals, and yeast. In industrialized countries, foods made with white rice or white flour are often fortified with thiamin (because most of the naturally occurring thiamin is lost during the refinement process).