...(PKU) test is done to check whether a newborn baby has the enzyme needed to use phenylalanine in his or her body. Phenylalanine is an amino acid that is needed for normal growth and development. If a baby's body does not have the enzyme that changes phenylalanine into another amino acid called tyrosine, the phenylalanine level builds up in the baby's blood and can cause brain damage, seizures, and intellectual disability. The damage caused by PKU can begin weeks after the baby has started drinking breast milk or formula. Babies with PKU need foods low in phenylalanine to prevent severe brain damage. Phenylalanine is found in most foods that have protein, such as milk, cheese, and meats. It is important to find this disease early. All babies in the United States and Canada are tested for PKU right after birth. To have the disease, you must inherit the gene from each parent. The United States Preventive Services Task Force recommends that all newborns be tested for PKU.1 The blood sample for PKU is usually taken from your baby's heel (called a heel stick). The test is done in the first few days after birth, as early as 24 hours after birth. The test may be repeated within the first week or two after birth. Why It Is Done A phenylketonuria (PKU) screening test is done to see whether a newborn baby has the enzyme needed to use phenylalanine in his or her body. If this test shows that your baby has a phenylalanine problem, the doctor will do further testing to check whether your baby has...
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...Phenylketonuria in Infants Introduction Phenylketonuria (PKU) is when a person cannot properly metabolize an amino acid known as phenylalanine that is found in food. The job of this enzyme is to chemically change the amino acid into other substances. When a child with PKU eats food containing phenylalanine, it builds up in the blood and causes problems phenylalanine is found in almost every food, except pure fat and sugar. If detected early at birth it can prevent mental defects for an infant. Phenylketonuria was the leading cause on infant death after birth before the 1960’s. Normally, such a rare condition would not attract such attention, but PKU is a treatable genetic disease. In the past, it generally resulted in severe mental retardation....
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...“Aspartame: Sweet Friend or Bitter Enemy?” Responding to the Public (Week 5 Alternative Assignment) HCS 539 What is Aspartame? “Human beings have an attraction to sweet food. Even ancient cave paintings at Arana in Spain exhibit a Neolithic man procuring honey from a bee’s nest. (Blachford, 2002). Aspartame is the name for a non-carbohydrate, non-nutritive artificial sweetener. It is synthesized from two amino acids, aspartic acid and the essential amino acid phenylalanine. It is the most commonly used sugar substitute on the market. It was first isolated and discovered in 1965 by James M. Schlatter. Schlatter was a chemist working for G.D. Searle & Company. It was an incidental finding while Schlatter was in the process of making an anti-ulcer drug. While turning a page in his reference materials, he licked his finger, only to find it incredibly sweet. During initial safety testing, there was some early debate as to whether aspartame was carcinogenic , or cancer causing, in laboratory rats. It was subjected to further studies before it was submitted to the FDA for approval as a food additive. Aspartame was finally approved for use, in dry foods, in 1974. Searle was initially not allowed to market it until 1981. In 1980, the FDA convened a board of inquiry into the claim that aspartame could have carcinogenic properties in rats, particularly brain cancer. The board’s findings were equivocal. They found that aspartame did not cause any documented...
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...Shikimic acid pathway Shikimic acid, more commonly known as its anionic form shikimate, is an important biochemical metabolite in plants and microorganisms. Its name comes from the Japanese flower shikimi from which it was first isolated. * Shikimic acid is a precursor for : * The aromatic amino acids phenylalanine and tyrosine * Indole, indole derivatives and aromatic amino acid tryptophan * Many alkaloids and other aromatic metabolites * Phenylpropanoids, flavonoids, tannins, and lignin. Biosynthesis of 3-dehydroquinate from phosphoenolpyruate and erythrose-4-phosphate DHQ is dehydrated to 3-dehydroshikimic acid by the enzyme dehydroquinase, which is reduced to shikimic acid by the enzyme shikimate dehydrogenase, which uses nicotinamide adenine dinucleotide phosphate (NADPH) as a cofactor. Biosynthesis of shikimic acid from 3-dehydroquinate The shikimate pathway is a seven step metabolic route used by bacteria, fungi, algae, parasites and plants for the biosynthesis of aromatic amino acids (phenylalanine, tyrosine, and tryptophan). This pathway is not found in animals and in humans, hence the products of this pathway represent essential amino acids that must be obtained from the animal's diet. The first enzyme involved is the shikimate kinase, an enzyme that catalyzes the ATP-dependent phosphorylation of shikimate to form shikimate 3-phosphate.[7] Shikimate 3-phosphate is then coupled with phosphoenol pyruvate to give 5-enolpyruvylshikimate-3-phosphate...
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...undergo further testing to determine if they are truly affected with a disease or if the test result was a false positive. Follow-up testing is typically coordinated between geneticists and the infant's pediatrician or primary care physician. Newborn screening debuted as a public health program in the United States in the early 1960s, and has expanded to countries around the world, with different testing menus in each country. The first disorder detected by modern newborn screening programs was phenylketonuria, a metabolic condition in which the inability to degrade the essential amino acid phenylalanine can cause irreversible mental retardation unless detected early. With early detection and dietary management, the negative effects of the disease can be largely eliminated. Robert Guthrie developed a simple method using a bacterial inhibition assay that could detect high levels of phenylalanine in blood shortly after a baby was born. Guthrie...
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...“Aspartame: Sweet Friend or Bitter Enemy?” Responding to the Public (Week 5 Alternative Assignment) HCS 539 What is Aspartame? “Human beings have an attraction to sweet food. Even ancient cave paintings at Arana in Spain exhibit a Neolithic man procuring honey from a bee’s nest. (Blachford, 2002). Aspartame is the name for a non-carbohydrate, non-nutritive artificial sweetener. It is synthesized from two amino acids, aspartic acid and the essential amino acid phenylalanine. It is the most commonly used sugar substitute on the market. It was first isolated and discovered in 1965 by James M. Schlatter. Schlatter was a chemist working for G.D. Searle & Company. It was an incidental finding while Schlatter was in the process of making an anti-ulcer drug. While turning a page in his reference materials, he licked his finger, only to find it incredibly sweet. During initial safety testing, there was some early debate as to whether aspartame was carcinogenic , or cancer causing, in laboratory rats. It was subjected to further studies before it was submitted to the FDA for approval as a food additive. Aspartame was finally approved for use, in dry foods, in 1974. Searle was initially not allowed to market it until 1981. In 1980, the FDA convened a board of inquiry into the claim that aspartame could have carcinogenic properties in rats, particularly brain cancer. The board’s findings were equivocal. They found that aspartame did not cause any...
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...|Student Name |Date | | | | |Questions |Student Response | | | | |Part I | | | | | |Original DNA Strand: |Transcription (base sequence of RNA): | | | | |3’-T A C C C T T T A G T A G C C A C T-5’ |UUC CCU UUU GUU GCC UCU | | | | | ...
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...What is the main feature of the topic? The main feature of this topic is the Reversible Addition-Fragmentation chain Transfer (RAFT) polymerization of N-acryloyl-L-phenylalanine (A-Phe-OH) where the carboxylics acid functional group is kept intact. Furthermore this is achieved without the use of a protecting group, such as methyl esters, as seen below. Figure 1: Preservation of Carboxylic Acid Moiety This article further examines the effect of chain transfer agents (CTA), solvents, temperature, and initiator molar ratio on the molecular weight distribution, and determines the optimal environment for direct polymerization. Why is this topic important? Carboxylic acid functional groups serve many purposes they confer to the polymer the ability to interact with a variety of substances. For example The use of amino based monomers with carboxylic acid moiety is the ability to adapt the polymers for various interactions with substances such as metal ions, non-ionic proton-accepting polymers, their derivatives, and cationic polyelectrolytes. As such these polymers can serve as a way of delivering drugs to targeted zones. Furthermore by polymerizing the monomers without the need for protecting groups serves a much more significant purpose on an industrial size. By avoiding the use of protecting groups one can avoid added steps in the industrial process, as such reducing the opportunities for errors to occur and avoiding additional costs associated with the protecting groups...
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...It was the year 1965 when chemist, James Schlatter was conducting tests for an anti-ulcer drug and unknowingly got some substance onto his fingers. Some time later that day, he was reading a book and was pleasantly surprised. when he licked his finger to turn a page, to notice a sweet taste. The substance he was working with was called aspartyl-phenylalanine methyl ester, phew what a mouthful! fortunately it is better known as aspartame! Each gram of aspartame contains four calories, sounds like a lot right? But since aspartame is roughly 200 times sweeter than sugar, less of it is needed to achieve the same sweet taste. Therefore it adds little to insignificant amounts of calories to food and beverages making it the perfect substitute to many other rival...
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...is born without the ability to properly break down an amino acid called phenylalanine. Described as an inborn error of amino acid metabolism, phenylketonuria (PKU) was the first genetic disorder found to be due to a specific enzyme deficiency, resulting in a patient's inability to metabolise a specific amino acid appropriately. Classical PKU is caused by a deficiency of the enzyme phenylalanine hydroxylase (PAH). Over 70 different mutations on the PAH gene found on Chromosome 12 have been found to cause the almost complete absence of PAH as seen in PKU patients. PKU patients deficient in PAH are unable to metabolise the amino acid phenylalanine leading to an accumulation of phenylalanine and it's metabolites within the body. PKU is an example of an autosomal recessive disorder. Causes Phenylketonuria (PKU) is inherited, which means it is passed down through families. Both parents must pass on the defective gene in order for a baby to have the condition. This is called an autosomal recessive trait. Babies with PKU are missing an enzyme called phenylalanine hydroxylase, which is needed to break down an essential amino acid called phenylalanine. The substance is found in foods that contain protein. Without the enzyme, levels of phenylalanine and two closely-related substances build up in the body. These substances are harmful to the central nervous system and cause brain damage. Symptoms Phenylalanine plays a role in the body's production of melanin, the pigment responsible...
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...Maria Rahim 0874389 BIMS 6322 – Gunasekera September 7, 2014 Abstract: Inborn Errors of Catecholamine Synthesis Catecholamines are an integral part of the pathway that relay neurotransmitters from the adrenal medulla of the brain to associated tissues within the body that can transmit or receive hormones and adrenergic receptors. Their biosynthesis begins with three aromatic amino acids: phenylalanine, tyrosine and tryptophan that lead to the production the catecholamines, dopamine and serotonin. Dopamine is further synthesized into norepinephrine and epinephrine. Devastating clinical features can arise from inborn errors of catecholamine synthesis which stem from three key regulatory enzymes within the pathway: GTP cyclohydrolase I, tyrosine hydroxylase and aromatic L-amino acid decarboxylase. Unlike its well known counterpart, phenylketonuria which arises from metabolizing deficiencies of...
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...poles. The final step of Meiosis is Telophase II. The cells divide and four haploids each with 23 chromosomes form. Mendel’s Law of Segregation states that everyone contains a pair of alleles which separate during cell division. The Law of Independent Assortment states that different kinds of genes for separate traits are passed from parents to offspring independently. The genetic disorder that I researched was Phenylketonuria. Phenylketonuria is an autosomal recessive metabolic disorder that affects the way a person’s body breaks down protein called phenylalanine. Phenylketonuria is caused by a mutation in chromosome 12. The gene within this chromosome codes for a protein called phenylalanine hydroxylase (PAH). PAH is an enzyme in the liver that breaks down the amino acid phenylalanine into other products. The shape of the PAH enzyme changes when the gene is mutated and then it is unable to break down phenylalanine. Phenylalanine builds up in the blood and poisons neurons in the brain. Therefore, if not treated Phenylketonuria can be destructive to the nervous system. This damage in the brain can result in intellectual disability....
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...Product History Aspartame * White powder * Made of L- aspartic acid, L-phenylalanine, methanol * 180 times sweeter than sugar * When exposed to high temperatures becomes unstable * Limited shelf life in soft drinks * Cost $85-$90 per pound * Accidently Discovered by G.D. Searle & Co. in U.S. Monsanto * Headquarters in St. Louis, Missouri * Leader in the US in production of agricultural products, plastics and specialty chemicals, performance materials and industrial control equipment * Summer of 1985 G.D. Searle was acquired by Monsanto Corporation * Searle developed markets for aspartame as a food and beverage additive * By 1986, Searle had built the NutraSweet to reach up to sales of $711 million. Tosoh Corporation * 1935 began as producer of soda ash and caustic soda * Since 1935 the company grown to become a diversified manufacturer of basic chemicals, intermediates, and downstream products, as well as scientific instruments and ceramics * Tosoh brought to the hook-up with DSM a patented process for manufacturing aspartame that employed a natural catalyst to solve the problem of achieving a precise coupling between the aspartic acid and phenylalanine inputs * Process was capable of using either L-Phenylalanine or D, L-Phenylalanine DSM * Based in Heerlen, the Netherlands * Company began as “Dutch State Mines” * Was a chemical group with interest in plastics, synthetic rubber, fine elements...
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...Threonine | 1834 mg | Isoleucine | 2293 mg | Leucine | 3259 mg | Lysine | 3689 mg | Methionine | 1203 mg | Cysteine | 556 mg | Phenylalanine | 1724 mg | Tyrosine | 1466 mg | Valine | 2155 mg | Arginine | 2619 mg | Histidine | 1348 mg | Alanine | 2369 mg | Aspartic acid | 3870 mg | Glutamic acid | 6504 mg | Glycine | 2133 mg | Proline | 1785 mg | Serine | 1494 mg | NAMES | mg | tryptophan | 406 mg | threonine | 1731 mg | isoleucine | 1898 mg | leucine | 3286 mg | lysine | 3577 mg | methionine | 1062 mg | cysteine | 445 mg | phenylalanine | 1621 mg | tyrosine | 1467 mg | valine | 2016 mg | arginine | 2566 mg | histidine | 1666 mg | alanine | 2263 mg | Aspartic acid | 3773 mg | Glutamic acid | 6157 mg | glycine | 1737 mg | proline | 1559 mg | serine | 1668 mg | hydroxyproline | 98.6 mg | List of Amino Acids presents in pork list of Amino Acids in chicken meat C.BEEF (PROTEIN-7.5 g D.MONGO (PROTEIN-3.2g) List of Amino Acids in beef list of Amino Acids in mongo NAMES | mg | Tryptophan | 118 mg | Threonine | 277 mg | Isoleucine | 310 mg | Leucine | 458 mg | Lysine | 465 mg | Methionine | 122 mg | Cysteine | 95.9 mg | Phenylalanine | 339 mg | Tyrosine | 284 mg | Valine | 432 mg | Arginine | 352 mg | Histidine | 166 mg | Alanine...
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...Phenylketonuria, Tay-Sach’s, and sickle cell anemia are all genetic disorders that affect many body systems; most cannot be cured. These genetic disorders can cause severe health problems that make it difficult to live a normal life. Health professionals can help treat or manage some of the symptoms to make them less severe. These disorders are caused by cell mutations and they cause severe damage to people’s body systems. Phenylketonuria is an inherited genetic disease caused by a mutation in the PAH gene. PKU causes increased levels of phenylalanine in the blood. Phenylalanine, obtained through diet, is a building block of amino acids. Left untreated, the levels of phenylalanine can build up and cause mild to severe health problems. Classic PKU is the name of the most severe form of the disease. Infants begin to show symptoms of classic PKU when they are a few months old. Children with classic PKU will develop permanent intellectual disabilities if left untreated. The most common symptoms are seizures, behavioral issues and developmental delay. Affected individuals might have a musty or mouse-like odor, lighter hair and skin than family members, and eczema. Although there is no cure for PKU, there are...
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