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…
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…
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…
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.
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.
Mrs. Danish, her teacher introduced Patty Pancake to her new classmates. “Good morning class, a new student has joined us today. Please…
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…
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…
Word Count: 1372
THE ‘B’ GRADE
Matthew S. Tapper loved the taste of maple syrup. He had no clue how it was made, and would salivate whenever he saw the ‘B’ grade pasted on a plastic half pint jug.
(Backstory: Not too many years back and for most of his millennial life when money flowed, the good Mr. Tapper went through gallon jugs of ‘B’ grade faster than the 4 or 5 at full throttle between 59th and 86th.)
Deep into his man purse he would dive for the errant coins that vacationed way…
Maple Syrup Urine Disease
Hannah Gentry , 13SK , 8/21/2014
Maple syrup urine disease (MSUD) is a rare genetic disorder where an aminoacidopathy secondary to an enzyme defect in the catabolic pathway of the branched-chain alpha-keto acid dehydrogenase complex, which are required in order to metabolize certain amino acids in the human body. In other words, it is a metabolism disorder, where the infant is unable to break down the amino acids: leucine, isoleucine, and valine. Build up of these…
Fenugreek has been used for healing a wide variety of symptoms and diseases for thousands of years. Some say that it was first discovered by ancient Romans who noticed that their cattle and horses seemed to prefer it to other forms of vegetation that was added to their fodder. Even the creatures that were sick and had no appetite would nuzzle through the hay to find pieces of it. Out of curiosity, physicians studied it to see what made it so special to the animals. And that was when they found…
Complex carbohydrates, like whole grains more often, to replace the spike-and-dip with a steady energy supply that keeps you going at full throttle.
Choose foods with more fibre and less added sugars.
Hidden Sugars: ...
Hidden sugars are commonly disguised as other ingredients.
Manufacturers add sugar to 74% of packaged foods sold in supermarkets.
There are at least 61 different names for sugar listed on food labels.
Here are some: Agave, Arabinose, Barley malt syrup, Barley sugar, Birch…