Maple Sugar Urine Disease Classic Maple Sugar Urine Disease or MSUD is a genetic amino acid disorder in which body isn’t able to break down the amino acids found in proteins such as leucine‚ isoleucine and valine. MSUD is also referred to as branched chain ketoaciduria. BCKADs or Branched-Chain Alpha-Keto Acid Dehydrogenase are enzymes that break down those amino acids. This causes the urine to smell like maple syrup. When these amino acids build up in the blood they cause complications.
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Alan Watson Mrs. Lefever Biology 19 April 2010 Maple Syrup Urine Disease I. General Information -Maple Syrup Urine disease is an inherited disorder in which the body does not and is unable to process certain protein building block called amino acids leucine‚ isoleucine‚ and valine. -To treat this disease the infected would have to have a protein-free diet. Fluids‚ sugars and possibly fats are given through a vein (IV). II. Nature of this Mutation -Symptoms of this
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Condition: Maple Syrup Urine Disease Maple Syrup Urine Disease is a genetic disorder caused by mutation in the DBT‚ DLD‚ BCKDHB‚ and BCKDHA genes‚ which codes for Branched Chain Keto Acid Dehydrogenase (BCKD). Normally‚ BCKD would process the keto acid derivatives for leucine‚ isoleucine‚ and valine and continue them on their respective metabolic pathways. As a result of these breaks in the metabolic pathway‚ the patient may suffer from mental retardation‚ lethargy‚ poor feeding habits‚ vomiting
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Maple Syrup Urine Disease Maple syrup urine disease or MSUD is a genetically inherited disease‚ characterized by a maple syrup odor found in the sweat‚ urine and earwax of affected individuals. This metabolic disorder was first documented in medical literature in 1954 by John Hans Menkes‚ a pediatric neurologist who would also discover Menkes Disease. People with this condition lack specific enzymes required to metabolize certain amino acids. It is also commonly known as BCKD deficiency‚ branched
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Huong Trinh Maple syrup urine disease Summary Maple syrup urine disease (MSUD) is an inborn error of metabolism which is caused by the branched-chain amino acid metabolism disorder. This disease happens when the body is unable to process certain protein blocks (amino acids) properly‚ then accumulate abnormally in the cells and fluids of the body‚ usually associated with newborn babies. Notably‚ the urine of patients often has a strong maple syrup odor. MSUD patients have inherited one
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Maple syrup was first collected and used by the indigenous peoples of North America. Vermont is the largest producer in the United States‚ generating about 5.5 percent of the global supply. Maple syrup is graded according to the Canada‚ United States‚ or Vermont scales based on its density and translucency. In the United States‚ a syrup must be made almost entirely from maple sap to be labelled as "maple". It is also used as an ingredient in baking‚ and as a sweetener or flavouring agent. Three
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Nature’s sweet maple treat Bobbie A. Harjo “Hurry up!” called John as Maggie grabbed the bucket in her trembling hands. She sprinted up the hill yelling‚ “I’m coming‚ I’m coming!” As she ran as fast as she could‚ she grew more and more worried. It was the last day of the sugaring season‚ and there were twenty more trees to harvest. Would we make it‚ she thought? When Pioneers like Maggie arrived to America‚ they learned how to make syrup. Maple syrup was first discovered by the Native
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doesn’t love pancakes smothered in maple syrup? The reason we have maple syrup with our pancakes or waffles is because of maple trees. This will tell you the ecological value‚ population density for the area‚ economic value‚ historical significance‚ use of photosynthesis‚ medicinal uses‚ building uses‚ and location in which the species grows. Ecological Value Maples trees are important to the environment and people for so many reasons. They produce maple syrup‚ so we can put it on our waffles or
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with severe lactic acidosis. Aminoacidopathies (e.g.‚ phenylketonuria‚ hereditary tyrosinemia‚ nonketotic hyperglycinemia‚ maple syrup urine disease [MSUD] and homocystinuria) may have similar presentation to the organic acidemias‚ but are a very heterogeneous group of disorders. Hereditary tyrosinemia can present in the neonate with a bleeding diathesis due to liver disease‚ or later in infancy with a renal Fanconi syndrome. The severe form of nonketotic hyperglycinemia presents as unremitting
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Top 10 list health benefits of pure maple syrup Maple syrup is a natural sweetener that can add to the health diet. It can make easy and stay away from the granular sugar and other sweetener‚ which is not healthy or unhealthy. The wholesale maple syrup can use for a salad dress on the roasted vegetable‚ granola and it’s fabulous oatmeal in the tea and other ways. The diet can give a great sweet kick being yummy‚ but they have some great health benefits. The high level of zinc and magnesium is helps
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