Maple syrup vitamins

Although predominantly sugar, maple syrup does contain some amount of several minerals and vitamins (Tables 4.7-4.8). A one-fourth cup serving contains 217 calories, but supplies 100% of the Canadian... 

TABLE 4.8 Vitamins in maple syrup (adapted from Perkins et al., 2006, used with permission)... 

Apeirt from children with thiamin-responsive maple syrup urine disease (Section 6.3.1.4) and thiamin-responsive megaloblastic anemia (Section 6.2), there are no established pharmacological uses of thieimin other than the treatment of deficiency. Because of the neurological involvement in thiamin deficiency, the vitamin has been used in nerve tonics, edthough there is no evidence that it has any effect except in cases of deficiency. 

Branched-chain ketoaciduria (commonly known as Maple Syrup Urine Disease MSUD) is another ailment that may be caused by thiamine deficiency. In MSUD, the oxidative decarboxylation of alpha-keto acids derived from, i.e. valine, isoleucine, and leucine, is blocked due to an inadequate supply of the coenzyme thiamine pyrophosphate (TPP). Clinical symptoms of MSUD include mental and physical retardation. Describe briefly the structure of Riboflavin (Vitamin B-2) and its biochemical role. 

Historically, a classic example of an evaporation process is the production of table salt. Maple syrup has traditionally been produced by evaporation of sap. Concentration of black liquor from pulp and paper processing constitutes a large-volume present application. Evaporators are also employed in such disparate uses as desalination of seawater, nuclear fuel reprocessing, radioactive waste treatment,preparation of boiler feed waters, and production of sodium hydroxide. They are used to concentrate stillage waste in fermentation processes, waste brines, inorganic salts in fertilizer production, and rinse liquids used in metal finishing, as well as in the production of sugar, vitamin C, caustic soda, dyes, and juice concentrates, and for solvent recovery in pharmaceutical processes. 

E. The correct response is very low levels of phoiylalanine hydroxylase, a key oizyme in the metabolic sequelae of phenylketonuria, that is, elevated phenylalanine, phoiylpyruvale, and para-hydroxyphenylpyruvate in blood. Homogentisic acid is an intermediate in the breakdown of tyrosine to fumarate and acetoacetate. Vitamin is required in the metabolism of branched-chain amino acids not phenylalanine. The a-keto acids of the branched chain amino acids produce the maple-syrup odor. 

Deficiency of methylmalonyl-CoA mutase results in methylmalonic acidemia, a condition in which methylmalonate accumulates in blood. The symptoms are similar to those of maple syrup urine disease. Methylmalonate may also accumulate because of a deficiency of adenosylcobalamin or weak binding of this coenzyme by a defective enzyme. Some affected individuals respond to injections of large daily doses of vitamin B12. 

The leucine-, isoleucine- and vafrne-free drink mix MSUD Aid III is suitable for infants, children, adolescents and adults but, unlike other proprietary preparations suitable for the management of Maple syrup urine disease, it is carbohydrate, fat, vitamin and mineral free, which need to be added to the diet. This may allow more appropriate age-specific supplementation including iodine. 

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