Maple syrup urine disease

Maple syrup urine disease is a rare disorder in which the body fails to properly process certain amino acids, such as leucine, isoleucine and valine. which are present in foods such as milk, eggs, meat. Therefore, results in a buildup of these in the blood. It is called maple syrup due to its distinctive sweet odor of affected infant’s urine. MSUD can damage the brain when in physical stress, such as fever, infection, starving. MSUD affects approximately 1 in 180,000 infants worldwide. A higher chance in children of Amish, Mennonite, and Jewish descent. Mutations in the BCKDHA, BCKDHB, and DBT genes can cause maple syrup urine disease. These three genes work together as a complex; they give instructions for the production of proteins. The…

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…

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…

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…

Madhavi Ramnath Genetics and Genomics Maple Syrup Urine Disease Maple syrup urine disease (MSUD), first characterized by John Menkes in 1954, is an inborn error of metabolism involving the catabolic pathway of the branched‐chain amino acids, where there is difficulty in breaking down certain parts of the protein (leucine, isoleucine, and valine). This leads to a buildup of chemicals in the blood and the build up of ketoacids, which give rise to the ‘maple syrup’ or burnt sugar smell in the…

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…

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…

Today I am going to teach you how to make a perfect thin, round, light pancakes. Pancakes are one of the most popular breakfast foods in America. We can thank the Ancient Greeks for this delicious delicacy. Back in the ancient times the GReeks used griddles to cook the pancakes and to this day griddles are still used. The way I make pancakes is a buttered square pan which is as easy as the griddle just with simpler steps. As these few steps fly by you will have a plate of heaven in front of you…

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…

Have you ever had maple syrup in the morning, with your either pancakes or waffles? Well if you have had maple syrup on anything, have you ever thought where it comes from? It comes from sap and that's what I am writing about. I picked this subject because I have always wondered where maple syrup came from. I will be writing about: what is sap, what are its uses, and why do trees produce sap. Sap is the fluid that gets carried through the xylem cells of a tree. Another name for sap,…