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[DOC File]RAJIV GANDHI UNIVERSITY OF HEALTH SCIENCES, …
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Maple Syrup Urine Disease (MSUD) is a genetic metabolic disorder resulting from the defective activity of the enzyme branched chain alpha-keto-acid dehydrogenase complex. Accumulation of the branched chain amino acids is toxic to the brain.
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[DOC File]1 - DCFirst
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4- Maple Syrup Urine Disease (MSUD) It is results from an absence or greatly reduced activity of the enzyme branched-chain keto acid decarboxylase, therby blocking the normal metabolismof the three essential branched-chain amino acids leucine, isoleucine, and valine.
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[DOCX File]AMCHOP - Pangasinan Chapter - HOME
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Maple syrup. Instructions. Mix the spelt flour, baking powder, baking soda, coconut sugar and cinnamon in a bowl. Add the almond milk, egg and vanilla and stir until combined; do not over mix. Place the bowl in the fridge for 15 minutes to set the batter. Place coconut oil in the pan and heat to medium heat.
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[DOC File]Nutrition is the science of food and encompasses ...
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Morbidity and mortality vary considerably. Some are relatively harmless (e.g. cystinuria and pentosuria), but some IMDs cause severe handicap (e.g. Phenylketonuria and Maple syrup urine disease) (1).Mental retardation is one of the important consequences of IEM. The incidence is estimated to be 1.5 - …
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[DOC File]Medical School Biochemistry - Fall 2001
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Sucrose ( glucose and fructose (beets, cane sugar, maple syrup) Lactose ( primarily found in milk and dairy products (glucose and galactose) Polysaccharides ( these are synthesized from glucose. Starch ( major digestible one and is increased when cooked. Amylose(Amylopectin ( increases blood glucose faster than amylase (easy to break down)
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[DOC File]Biochemistry Related to Clinical Chemistry
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Branched-chain -keto acid dehydrogenase complex. Maple syrup urine disease (MSUD)/branched-chain ketonuria. Diet restriction, branched-chain keto acids supplement. Phenylalanine and tyrosine. Phe ( Tyr: phenylalanine hydroxylase and phenylketouria (PKU) The …
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[DOCX File]Welcome – THE ORGANIC, VEGAN, GLUTEN FREE, DAIRY …
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Third, the keto acids did not show a need for preceding oximation. ... enantioselective capillary GC–MS was applied for analysis of the main uri-nary metabolites excreted at maple syrup urine ...
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Keto Maple Syrup - Maria Mind Body Health
Maple syrup urine disease (MSUD) The normal metabolism of the branched chain amino acids Leucine, Isoleucine, and valine Valine. (Val) (V) is glucogenic; Leucine (Leu) (L) is ketogenic while Isoleucine (Ile) (I) is both ketogenicand glucogenic. All the three are essential amino acids.
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[DOC File]Inborn error in metabolism of amino acids
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Maple syrup urine disease. Phenylketonuria. Tyrosemia. Ketohexose. Pantothentic acid is necessary for production of? Coenzyme A. Amino acids. Cobalamine. Cytochrome B. As the density of lipoproteins is increased ? decreases and ? increases? Protein, sterol. Tricylycerol, sterol. Triacylglycerol, proteins. Protein, triacylgycerol Which is ...
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[DOC File]Review on Biochemistry: Protein Chemistry
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with the result that homeostasis is maintained. Which of the following statements about. the regulation of metabolic pathways under high energy charge conditions is
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Is maple syrup keto