Hereditary fructose intolerance Essay Example for Free

Hereditary fructose intolerance Essay Enzymes are proteins that increase the rate of chemical activity. Their three dimensional structure determines their function, and is made of chains of amino acids that have folded into a specific shape with a unique property. Enzymes lower the activation energy needed for a chemical reaction, therefore speeding up the process. Multiple enzymes work together in metabolic pathways, taking a product (end result) of one metabolic reaction as a substrate (substance or molecule at start of process) for another reaction. Metabolic pathways create the avenue for fructolysis, the breakdown (catabolism) of fructose, occurring in the liver, and in muscle and fat tissue. Most of fructose obtained by diet is metabolized in the liver, where the enzyme fructokinase is abundant. This enzyme phosphorylates the substrate (fructose) into fructose-1-phosphate, which is then split into glyceraldehyde and dihydroxyacetone phosphate, both three carbon molecules that can enter the glycolytic pathway for further oxidation and energy production. Enzymes are specific to a substrate, meaning they have an active spot on the enzyme that will only work with a specific shape of a particular substance (substrate). Aldolase B deficiency Aldolase B is an enzyme found predominantly in the liver that plays a major role in fructose metabolism. In hereditary fructose intolerance (HFI), individuals have a build up of fructose 1- phosphate (F1P), due to the absence or deficiency of aldolaseB, necessary to break down F1P into the two three carbon molecules utilized in the glycolytic pathway. The F1P is toxic to cells and tissues in the body, causing phosphate to become unusable, and depleting phosphate storage and energy. The decreasing phosphate levels cause glycogenolysis in the liver to halt, affecting blood sugar levels. Persons with HFI can exhibit symptoms of vomiting, trembling, nausea, lethargy, hypoglycemia, hepatomegaly (enlarged liver), and jaundice after consuming foods containing fructose. These persons must avoid fructose to avoid the build up of F1P in their body, having a deficiency in aldolase B needed to metabolize the F1P. Fructose 1-phosphate Fructose is a monosaccharide, and has the same chemical formula as glucose, but a different structure. Fructose, a simple sugar found in honey, fruits, or even table sugar, is phosphoylated in the liver by the enzyme fructokinase, or in cells by enzyme hexokinase, to form fructose 1-phospahate. Adenosine triphosphate (ATP) supplies the phosphate group for both reactions. Aldolase B further breaks down the product (F1P) of fructolysis, (similar to glycolosis, just with fructose, instead of glucose) into two trioses. Role of aldolase B in breakdown of fructose Aldolase B is located mainly in liver of body and ensures second part of fructose metabolism is carried out. Aldolase B breaks down fructose-1-phosphate into two trioses, glyceraldehyde and dihydroxyacetone phosphate (USNLM, 2012). Both of these three-carbon molecules are needed for further enzymatic processes in our bodies. Case 2- Mitochondrial disease Mitochondrial disease is a complex failure of mitochondrial functions. Mitochondria supply most of the energy utilized in the body, and when not functioning properly, decreased energy production cause system failure that create cell injury and cell death. The person with this affliction faces serious health concerns, as there is no cure for mitochondrial disease, only symptom management. Cori cycle The Cori cycle occurs in liver cells, cells lacking mitochondria, and is important part in anaerobic glycolysis. Pyruvate, a product of glycolysis, is converted to lactate through anaerobic respiration. Anaerobic glycolysis produces lactate from breaking down glycogen in muscles. The lactate produced in skeletal muscle by anaerobic glycolysis is transported to liver after being released into bloodstream, for conversion to glucose. Then glucose is returned to muscle in blood for energy and glycogen replenishment (King, 2012). Glucose being consumed and resynthesized at the expense of ATP and GTP hydrolysis is termed by scientists as a â€Å"futile cycle† because it takes more energy (ATP) than is produced, with a net loss of 4 ATP (Wiley, 2012). This indicates that if the conversion of lactate to glucose occurred in same cell, the energy reserves of the cell would be depleted, because the 2 ATP produced by glycolysis would be offset by the 6 ATP needed for a gluconeogenesis, to convert lactate into glucose for muscle replenishment. This continued cycle would cause an energy deficit, as the muscles glycogen stores are minimal, so the energy reserves would be depleted rapidly. Citric acid cycle During step 5 of the citric acid cycle (Krebs cycle), a phosphate is bonded to the succinyl complex, once containing coenzyme A. This phosphate is then transferred to a GDP molecule, to be converted into a GTP molecule. This GTP molecule will give a phosphate to ADP to make an ATP for energy. A defect in this step would prevent an increased conversion of ADP to ATP when energy needs rise. Initially, acetyl CoA combines with oxaloacetate to make a citrate molecule. The citrate is isomerized to form isocitrate, which then is oxidized by NAD. This creates an unstable molecule that releases a CO2 molecule, creating alpha-ketoglutarate. Now, the acetyl CoA that was released in first step returns to oxidize the alpha-ketoglutarate and initiate conversion to succinyl-coenzyme A complex. A free H2O donates its hydrogen to the coenzyme A, and a free phosphate comes in to replace the coenzyme A. This newly bonded phosphate will be transferred to GDP molecule as indicated previously, for conver sion to GTP, then ADP, and finally ATP for energy. Coenzyme Q10 Coenzyme Q10 ( CoQ10) is found in cell membranes, and is critical in all cells in producing ATP (energy) for the body. CoQ10 carries electrons from enzyme complex I and II to complex III in mitochondria. This transfer of electrons in the electron transport chain (ETC) pushes hydrogen through the inner cell membrane to make a proton gradient needed by ATP synthase to make ATP (Wikipedia, 2012). CoQ10 is the lone molecule that does this task in ETC, and is vital to this function of ATP production in cells. References King, Michael W, PhD. (2012). Gluconeogenesis: Glucose synthesis. Retrieved from The Medical Biochemistry Page website: http://themedicalbiochemistrypage.org/gluconeogenesis.php U.S. National Library of Medicine. (2012). ALDOB. Retrieved from http://ghr.nlm.nih.gov/gene/ALDOB Wikipedia. (2012). Coenzyme Q10. Retrieved from http://en.wikipedia.org/wiki/Coenzyme_Q10 Wiley, John. (2012). Interactive Concepts in Biochemistry: The Cori Cycle. Retrieved from http://www.wiley.com/college/boyer/0470003790/animations/cori_cycle/cori_cycle.htm

Cantebury Tales - Relation Of Wife Of Bath To Contemporary Women Essay

Hundreds of centuries before the fourteenth century, during it and yet still after, civilization, led by the educated theologians, politicians and whoever else made up the ruling class, women were looked at as the Devil’s ally – a sensual and deceitful creature who was a constant bearer of sin and the cause of most of man’s misfortune. Women then and now may look upon most of these â€Å"devilish† characteristics as desirable, strong-willed and feministic. Chaucer appears to support women and specifically these devilish feminists by creating two very strong-willed and successful women in the Wife of Bath and the old hag in the Wife’s tale. However, through all of the tough outer attributes, on the inside are the same classic and traditional damsels in distress that require a man just like the women of that time always had. Through the original strong qualities of the two women, Chaucer provides a hopeful example and model for women of now and then. Furthermore, by giving these women some stronger, domineering and â€Å"masculine† features Chaucer is terrifically satirizing the gender roles and stereotypes of the time. Along with all of these strong feminist messages also come out anti-feminism ideals about keeping women in a certain role, causing a lengthy and intelligent debate upon what Chaucer really meant. All of these reasons are why it is important to discuss and understand The Wife of Bath’s relation and influence on contemporary women. Chaucer’s main target of his satirical wit and criticism throughout his Canterbury Tales is the Anglo-Saxon church and even though in this tale he focuses more on the gender debate his fiery scorn and contempt of the corrupt church and its disciples is embodied in the Wife’s prologues first three lines: â€Å"Experience, though no authority, Were in this world, were good enough for me, To speak of woe that is in all marriage;† Here Chaucer, through the eyes of a women, points out that there is far too much reliance on authority, meaning the opinions of older and perhaps ancient writers. This sort of authority was responsible for the horrible distortion of woman’s character and place in society and thus Chaucer felt his satirical and sarcastic attack about love in marriage was necessary. Chaucer does it through the Wife of Bath as a medium to reach the hopelessly ignorant women of the time should they hear of the tale. The Wife o... ...When he had leisure and took some vacation, From all his other worldly occupation, To read, within this book, of wicked wives.† His reading of â€Å"wicked wives† and clearly anti-feminist literature is a perfect example of she no longer having control as is her husbands statement: â€Å"A woman fair, save she be chaste also, Is like a ring of gold in a sow's nose.† By this he means a fair and good woman is no more than an ornament or accessory to her man. Clearly if he was being mastered by a woman he would not make statements such as these. The Wife of Bath, Dame Alice, portrays and exhibits numerous qualities of a strong and confident woman and an unknown feminist. Furthermore, these characteristics are what women nowadays point to as Chaucer’s bravery to point out and criticize the unfair treatment of women over the centuries and eras. Unfortunately for them, Chaucer was not quite as brilliant and innovative as contemporary women would like to think. As evident with the hypocrisy showed by the Wife of Bath. In conclusion, The Wife of Bath definitely has some strong positive messages for contemporary women and even yet he deeper shows them what not to do with the hypocrisy of the Wife.

I Really Enjoy Family Time.

Griselda Soto English 99 Dennis Pearson 04/16/13 I really enjoy family time. Family is not an important thing, it’s everything. There are people who put work first, or anything else before their family. I personally think there is nothing more important than family. Because, what is the purpose to live just to work? In the end of our lives, we are not going to be able to take anything with us. There is nothing else I enjoy more than spending quality time with my daughters.I believe that by spending quality time with them, I can take advantage of the situation and teach them good values. Even I learn a lot from them because they always teach me the importance of appreciating little things in life. One of the things I love the most from them is that they always remind me that I have to live life to the moment. Unlike most adults, children find joy in almost everything as riding a bike, reading a book, or even by drawing a picture. That is one of the main reason I enjoy so much s pending time with my daughters. They always give me joy.When I see My daughters smile it's when I know they are having fun, for me that worth more than all the money in the world. My daughters always amuse me and fill me with joy. They taught me that no matter what you do, as long as you use your imagination everything could be fun. So I always do my best to put in first place what really matters in my life because life goes in the blink of an eye. That's why I put first what is truly meaningful in my life. I have learned a lot from my daughters, for example: To always take advantage of any situation.

The Ron Clark Story . Summer 2016, A Time Where I Was At

The Ron Clark Story Summer 2016, a time where I was at my worst and in a hard place. I was in a terrible motorcycle accident that left me injured and feeling like I’d lost hope. This was the summer before I began my first semester in the blocks program. I was constantly debating on if I wanted to start the semester or if I should take a semester off to heal. It wasn’t until one evening my mom came home and was talking about this amazing seminar she went to that afternoon. Her first statement was on how inspirational the speaker was. She shared with me the story about a man who went into a Harlem school and completely changed the lives of a group of students who everyone had given up on. The guest speakers name was Ron Clark. The story†¦show more content†¦Clark wasn’t afraid of a challenge and demanded to be put within this classroom. Before starting his first day in the classroom Clark went to all of the homes of his students to introduce himself to the parents and to get a feel of where his students were coming from. When he went to one of the homes of a female student within his class named Shamika, he saw that she was home alone taking care of her three siblings. She cooked, cleaned, and made sure her siblings were cared for. This student in the film reminded me a lot of one of my students in my Tuesday field. She has multiple siblings and her mother requires her to help take care of them. Shamika is one of the main students within his class that he has a lot of trouble with in the beginning but creates a strong relationship with in the end. The process that Mr. Clark goes through to get his students in his class to respect him as a teacher was tough. His students just saw him as another teacher who was going to give up and leave them. Clark set out to make sure that he didn’t leave he wanted to form a bond with his students. He set rules for the class but they quickly saw all of the rules as one giant joke. It wasn†™t until one day that Clark couldn’t take the fact that his students didn’t see him as a teacher figure and he just walked out of the class for the day. The students then saw that he gave up on them like the rest of the teachers. But the next day Clark was back inShow MoreRelatedProject Managment Case Studies214937 Words   |  860 Pagesfitness for a particular purpose. No warranty may be created or extended by sales representatives orwritten sales materials. The advice and strategies contained herein may not be suitable for your situation. You should consult with a professional where appropriate. Neither the publisher nor author shall be liable for any loss of profit or any other commercial damages, including but not limited to special, incidental, consequential, or other damages. For general information on our other products andRead MoreExploring Corporate Strategy - Case164366 Words   |  658 Pagestogether with important subsidiary foci (where appropriate). I n general, the sequence of cases is intended to mirror the chapter sequence. However, this should not be taken too literally because, of course, many of these cases cover a variety of issues. The ‘classiï ¬ cation’ provided is therefore guidance only. We expect readers to seek their own lessons from cases, and tutors to use cases in whichever way and sequence best ï ¬ ts the purpose of their programmes. Where cases have been chosen to illustrateRead MoreCase Study148348 Words   |  594 Pagesand Scholes, Exploring Strategy, 9th Edition, Instructor’s Manual Ryanair: The Low Fares Airline–Future Destinations? Will We Still Love IKEA? CRH plc: Successful Corporate-level Strategy in a Challenging Environment SABMiller Marks Spencer: Where Next for the Icon of British Retailing? Tesco: from Domestic Operator to Multinational Giant Ekomate Systems and the Indian Software Industry: Leveraging Network Relationships for International Growth Sustaining the Magic at Bang Olufsen Cordys: