Maple syrup urine disease

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    group of rare disorders characterized by metabolic pathway abnormalities. One such disorder is Maple Syrup Urine Disease (MSUD) otherwise known as Branched-chain Ketoaciduria, a dangerous condition in which over saturation of amino acids in the blood can lead to toxicity progressing to encephalopathy, neurodegeneration, coma or respiratory failure (Haldeman-Englert, 2015). MSUD results from a malfunction of the branched-chain alpha-ketoacid dehydrogenase complex (BCKD) (Kniffin, 2013). This complex produces enzymes necessary for the proper breakdown of common amino acids such as valine, leucine, and isoleucine, are found most abundantly in protein rich foods (Strauss et. al, 2013). When the aforementioned amino acids are not broken down they remain in the blood along with their keto acids leading to acidemia (Strauss et. al, 2013). As discussed by the National Organization for Rare Disorders (NORD) MSUD is caused by an autosomal recessive mutation, indicating that the…

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    Maple Syrup Urine Disease (MSUD) is a rare, autosomal recessive metabolic disorder in which the body is unable to process certain amino acids properly. It causes brain damage and progressive nervous system degeneration. MSUD is caused by mutations in three different genes: BCKDHA, BCKDHB and DBT (Warrell, Cox and Firth, 2012). The BCKDHA gene causes MSUD Type Ia due to the mutation in the E1α subunit, BCKDHB causes Type Ib due to the mutation in the E1ᵦ subunit, and DBT causes Type II due to…

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    Biochemistry in the Biotech Industry Maple Syrup Urine Disease: A Sweet Scented Killer Maple Syrup Urine Disease (MSUD) is a rare, potentially deadly disorder where the body cannot break down the three branched- chained amino acids (BCCA): leucine, isoleucine and valine. These amino acids are used to build proteins and when they are not being used they can be broken down and recycled in the cell. The complex that breaks these amino acids down is called BCKD (branched-chain alpa-ketoacid…

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    Tree of Heaven is a sumac plant that is native to China, but it moved into Indiana and is now an invasive species. It’s scientific name is Ailanthus altissima. The tree of heaven came to the United States in the late 1700’s. The reason that it’s an invasive species is because it crowds out native species and damages pavement and building foundations. Tree of heaven is a native of China and in 1751 was mistakenly introduced into France and England by a missionary who though it was a Japanese…

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    Allegory of the Maple Syrup April 12, 2010 at 6:53am Throughout His ministry, Christ used stories and metaphor to teach the people the gospel. On the surface, the stories are just that-- stories. Some were allegories. An allegory is a Greek word. It means a figurative story full of meaning and metaphor, where each thing in the story represents something else. If you look deeper into the meanings and symbols, bright truths can be learned. No matter what level a person is on, we can all gain light…

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    Suger Maple Research Paper

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    Suger maple(Acer saccharum) Aceraceae/ Maple family sometimes called rock maple or hard maple, it is one of the largest hardwoods that is wide spreaded in western Canada. Sugar maple is common associtated with those common types: Acer negundo,manitoba maple Acer ginnala,amur maple Acer sacharrinum,silver maple Tree with overall up to 35 m tall, long straight trunk, usually branch-free for 2/3rds of its height. Leaves: Simple leaves, oppositely arranged and they’re flat, with three or five…

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    Patty Pancake Speech

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    Page 1 Once upon a time in a kitchen not so far away, there lived in a small cottage made of milk and honey and all things yummy, Patty Pancake, Mommy Muffin and Daddy Doughnut. Page 2 Patty Pancake was so excited to begin her first day of school at Maple Syrup elementary. Telling Mommy Muffin and Daddy Doughnut about all of the friends she would meet. Page 3 Mrs. Danish, her teacher introduced Patty Pancake to her new classmates. “Good morning class, a new student has joined us today. Please…

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    Vermont. My mom, dad, sister, and two dogs pile into the car at the break of dawn to travel. We take this weekend trip to visit our beloved grandmother, Mimi. It is always a pleasure visiting her, but the car ride is always so long. Miles after miles, there seems to be no end. There is only one thing stopping me from losing my mind during this ride, maple creemees. The taste lingers in my mouth the entire weekend, the taste of the cream as it melts onto my tongue and slowly freezes every…

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    I asked my leaders if I could say hello to Gabby who was just down the hill, but I was told no. Our lunch break ended and it was time to go back to work. Later two of the girls had to leave so me and another girl filled in for them. Soon we got maple sugar ice cream. It may have been freezing but dang that ice cream with the maple syrup was good. It had a cold sweet kind of taste.The sky started to get darker as we were finishing up with our last group. Eventually girls from other groups had to…

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    Cayuta, N.Y. (WENY) -- This winter's unseasonably warm weather is creating a change for maple syrup producers. The shift in temperatures has many farmers unsure of when or how long their tapping season will last. Even though many syrup producers begin to tap their trees in mid-February to mid-March, this season's unpredictable weather have put maple producers in a sticky situation. "The weather this year has us kind of scrambling. With some of the recent storms, and a lot of wind, we've…

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