...reaction rates without changing the chemical equilibrium between the reactants and products. ( Bhagavan et,al 2006). * The reaction between two substrates are catalyzed by enzymes. The enzyme brings a template upon which the two substrates are combined together in the proper position and make them to react each other. Deficiency in aldolase B and hereditary fructose intolerance * Hereditary fructose intolerance is a condition I which affects a humans ability to digest the fructose sugar. The incidence of hereditary fructose intolerance is 1to 2 in 20000 to 30000 individuals in a year worldwide. .( John .R.H 1996) * Hereditary fructose intolerance can be caused by mutations in the ALDOB gene. The ALDOB gene is responsible for making the aldolase B enzyme. The aldolase B enzyme is primarily seen in the Liver. This helps for the fructose metabolism. This enzyme is responsible for the further step in the metabolism of fructose, which breaks down the molecule fructose-1-phosphate into other molecules called glyceraldehyde and dihydroxyacetone phosphate. * The lack of aldolase B can results in the accumulation of fructose 1 phosphate in the liver. This seems to be toxic and can cause death of liver cells. The short of aldolase B can cause the reduction of dihydroxyacetone phosphate and this will lead to decrease in phosphate level in the body. ( Monique L 2008) * The damage...
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...2013) Every enzyme has an active site where the substrate binds. Once bound to the enzyme, it breaks sown the substrate and releases it as product. The enzyme remains intact and ready to repeat the process. (Ahern, p.4-6) What happens in inherited fructose intolerance? Aldolase B is the enzyme needed to break down fructose. When someone without aldolase B consumes fructose or sucrose their body cannot change glycogen (energy storage material) to glucose. Because of this the blood sugar levels fall and toxins collect in the liver. This condition is hereditary, meaning that it is passed down from parents whom either exhibit this condition or is a carrier of it. (UMMC, 2011) . . . Fructose-1 phosphate is the substrate that functions in fructose metabolism. Without the activity of adolase B, fructose-1 phosphate rapidly collects in the liver. This activates AMP deaminase which secludes adenine nucleotides causing hypoglycemia. (Tolan, 2013) What then? After fructose is absorbed, it is phosphorylated by fructokinase forming fructose-1 phosphate (F-1P). Aldolase B then breaks down F-1P to glyceraldehyde and DHAP. After glyceraldehyde is phosphorylated by triose kinase forming G3P, both products can be changed to...
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... occur without a catalyst, but with the catalyst it will speed the process up. • Carbohydrates will be broken down into sugar on their own but when you add a enzyme to the process (like your saliva) it will speed up. You also won’t use up as much energy in the process. A2 If Aldolose B is not present this is called hereditary fructose intolerance. If there is a deficiency of aldolase B it will cause an accumulaKon of F1P and will “trap” the phospahte. Fructose, once ingested, is broken down to fructose-‐1-‐phosphate(F1P) by the liver , by fructokinase. F1P is metabolized by aldolase B. As the process tries to move along the enzyme is blocked and there is a reduced amount of ATP producKon and inhibiKon of glucose. If aldolose B is not present then their will be an...
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...04-07 A. Case 1: Hereditary Fructose Intolerance A1. Role of Enzymes in Processes Enzymes are proteins that carry out chemical reactions. They bind to substrates, which are basically substances that need to be broken down and changed into something else. When the enzyme and substrate bind, they form the enzyme-substrate complex. An enzyme will act in a specific way on the substrate that it is bound to in order to change it into a product, and at the end of the process, the enzyme is unchanged and ready to bind to the next substrate. An enzyme acts as a catalyst, something that lowers the energy required to complete a chemical reaction (activation energy) without itself being changed. (Hudon-Miller, 2012) In the case of fructose breakdown, an enzyme called fructokinase is responsible for splitting fructose into fructose 1-phosphate, a six-carbon fructose. Another enzyme called aldolase B splits fructose 1-phosphate into two three-carbon molecules, dihydroxyacetone phosphate (DHAP) and glyceraldehyde. These products are then able to enter the glycolysis pathway to be converted to pyruvate, which is essential for the citric acid cycle and the production of adenosine triphosphate (ATP) for cellular energy. A2. Deficiency in Aldolase B A hereditary deficiency in aldolase B could be caused by mutations in the ALDOB gene. An aldolase B deficiency will prevent the breakdown of fructose past the point of the fructose 1-phosphate stage. This causes fructose-1-phosphate to build...
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...04-07 A. Case 1: Hereditary Fructose Intolerance A1. Role of Enzymes in Processes Enzymes are proteins that carry out chemical reactions. They bind to substrates, which are basically substances that need to be broken down and changed into something else. When the enzyme and substrate bind, they form the enzyme-substrate complex. An enzyme will act in a specific way on the substrate that it is bound to in order to change it into a product, and at the end of the process, the enzyme is unchanged and ready to bind to the next substrate. An enzyme acts as a catalyst, something that lowers the energy required to complete a chemical reaction (activation energy) without itself being changed. (Hudon-Miller, 2012) In the case of fructose breakdown, an enzyme called fructokinase is responsible for splitting fructose into fructose 1-phosphate, a six-carbon fructose. Another enzyme called aldolase B splits fructose 1-phosphate into two three-carbon molecules, dihydroxyacetone phosphate (DHAP) and glyceraldehyde. These products are then able to enter the glycolysis pathway to be converted to pyruvate, which is essential for the citric acid cycle and the production of adenosine triphosphate (ATP) for cellular energy. A2. Deficiency in Aldolase B A hereditary deficiency in aldolase B could be caused by mutations in the ALDOB gene. An aldolase B deficiency will prevent the breakdown of fructose past the point of the fructose 1-phosphate stage. This causes fructose-1-phosphate to build...
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...alter chemical reactions by decreasing the rate at which reactions occur and its by-products causing an increase demand for energy. When there is a defect in enzyme production it can severely alter how our bodies metabolize certain substances. Such as fructose, this is metabolized in the liver, and broken down by an enzyme called fructokinase. As fructose is broken down by fructokinase, it is converted to fructose 1-phosphate. The process continues as fructose 1-phosphate (f-1-p) is catalyzed by aldolase-B into DHAP-glyceraldehyde. The products generated by these reactions is formed into ATP, and used as cellular energy or when not needed, it is stored as glycogen in the liver for later use. If aldolase B were absent it would lead to a condition called, Hereditary Fructose Intolerance (HFI). HFI, an autosomal recessive disorder, occurs when aldolase-B does not function properly. Fructose 1-phosphate if not catalyzed by aldolase-B will cause a disruption in the cycle as the conversion of DHAP and glyceraldehydes are not produced; therefore glycolysis and glycogenesis cannot occur reducing ATP synthesis. Severe hypoglycemia and possible liver failure becomes present in this condition due to fructose intolerance. When fructose is converted to f 1-p, and is not further broken down by aldolase-B; glycolysis, and glycogenesis do not occur impairing liver...
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...(lock and key or induced fit model). A3. Create a diagram that illustrates the aE of a reaction in the presence and absence of an enzyme. A4. Explain the reactions catalyzed by enzymes in the first two steps of fructose metabolism in the liver. Fructose in the blood passes through the cell membrane into the liver cell and initiates phosphoralation with fructokinase for the metabolism of fructose. The fructokinase then uses the phosphate and produces F-1-P (fructose 1 phosphate). F-1-P is the substrate for the enzyme Aldolase B. Aldolase B takes the F-1-P and makes DHAP and glyceraldehyde, the products of Aldolase B. The DHAP and glyceraldehyde are intermediates in glycolysis and continue down to make pyruvate which then can make ATP synthesis or fatty acids. (Sanders, J. 2013) A5. Discuss how a deficiency in aldolase B is responsible for HFI. Hereditary Fructose Intolerance (HFI) results from a deficiency of aldolase B activity primarily in the liver, but also in the kidneys and small intestine. If aldolase B doesn’t work, then there is no effect on fructokinase, there is no effect on the enzyme that separates glucose and fructose and no effect on the enzymes working on glycolysis. Only aldolase B is used. So, the fructose still gets converted to the fructose-1-phosphate, but without aldolase B, it cannot be converted to DHAP and glyceraldehyde. The lack of the aldolase B enzyme...
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... Once they find a match the enzyme takes the substrate and creates a product out of it. The product then continues on the pathways to be a substrate for a different enzyme. ¨. If the temperature is up, enzymes work faster. ¨. If the temperature is down, enzymes work slower. ¨. No work occurs if the temperature is too hot. ¨. Aldolase B is an enzyme that breaks fructose down. (Enzyme Characteristics) Aldolase B & HFI ¨. Aldolase B turns Fructose-1-Phospate to DHAP andglyceraldehyde. These two substances then enter glycolysis to make ATP for the body. ¨. If Aldolase B is lacking, fructose can not be broken down, potentially causing Hereditary Fructose Intolerance. ¨. In this process, there is a protein that is missing that breaks down fructose. Glycogen is not broken down and Fructose-1-Phosphate builds up on liver as well as kidneys. ¨. With fructose not being broken down, hereditary fructose intolerance occurs. ¨. Symptoms are: pain in abdomen, vomiting, as well as hypoglycemia after consuming fructose broken down through F1P ¨. In babies, this process can lead to hepatic/renal...
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... chemical reaction or products (HudonMiller, 2013). A2. BIOCHEMISTRY TASK 4 2 A3. A4. Fructose metabolism has two steps different steps in the liver, the substrate of fructose breaks down to fructose1phosphate by an enzyme called fructokinase; fructose1phosphate is turned into DHAP + glyceraldehyde by an enzyme known as aldolase B, DHAP +glyceraldehyde BIOCHEMISTRY TASK 4 3 A4 continue then enters glycolysis, glycolysis turns into pyruvate goes through citric acid cycle and produces ATP (HudonMiller, 2013). BIOCHEMISTRY TASK 4 5 A5. In hereditary fructose intolerance there is an aldolase B (which is the enzyme for the substrate fructose1phosphate) can no longer take its substrate fructose1phosphate and turn it into products like glycogen, fatty acids synthase or ATP synthase (Sanders, 2013). Therefore, with the lack of aldolase B the fructose is still being broken down into fructose1phosphate by the fructokinase and causing a build up of fructose1phosphate and it can not enter glycolysis or gluconeogenesis (Sanders,...
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...enzyme has a receptor site, and they are very specific to which molecule (substrate) it will interact with. When a substrate is captured, it will either be combined to create a product or it will be broke down. Fructose is primarily metabolized in the liver. Fructose alone cannot be used as energy. It has to be broke down for use. Enzymes in the liver aid fructose metabolism. Fructose binds to the receptor site on the enzyme fructokinase. This enzyme uses ATP and ADP cycle (energy) to speed up the chemical reaction to convert fructose into Fru-1-p. Next, Fru-1-p will undergo the next reaction and will produce either DHAP or glyceraldehyde by way of the enzyme Aldolase B. (Wikipedia, 2015) As stated above, Aldolase B is active specific to the substrate Fructose-1-Phosphate. Fructose-1-phos is derived from fructose. It’s produced by fructokinase which is available in the liver. It’s converted by aldolase B into dihydroxyacetone phosphate and glyceraldehyde. A deficiency in this enzyme (Aldolase B) caused a disorder called Hereditary Fructose Intolerance. This is a genetic mutation of chromosome 9 which is an autosomal recessive disorder, meaning that the trait must be passed from both parents. The mutation in Adolab gene results in hereditary fructose intolerance (HFI). Aldolab is accountable for creation of the Aldolase B enzyme. A deficiency in enzyme...
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...help break up large molecules faster than if there were no enzymes, thus releasing energy into the body faster. One very important enzyme in the human body is aldolase B, it is responsible for the metabolizing fructose. Fructose is a naturally occurring monosaccharide in the body. Fructose often bonds with sucrose and becomes what most people know as table sugar, which is a disaccharide. (United States National Library of Medicine, 2011). Before fructose is broken down to be used as energy, is it converted to fructose-1-phosphate. As mentioned earlier, aldolase B is needed to break down fructose in the human body. If there is a deficiency of aldolase B, a person will experience Hereditary Fructose Intolerance (HFI) HFI is an autosomal recessive trait that is related to a mutation of the ALDOB gene (USNLM, 2011). This gene is responsible for making the aldolase B enzyme. The mutated gene causes the aldolase B enzyme to be misshaped, unable to function properly. When a person ingests fructose, it is converted to fructose-1-phosphate. After this conversion, fructose-1-phosphate should be metabolized into aldoase B. When there is a deficiency in aldolase B, the fructose is signaled by the fructose kinase to keep making fructose-1-phosphate. Along with this deficiency, fructose-1-phosohate is no longer being used as a substrate to make the products DHP and glycol aldehyde, which allows even more...
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...and are involved in the process of fructose breakdown. The enzymes are catalysts, meaning, they work to lower activation energy without using the reaction. Sucrose is plain table sugar and it is broken down into glucose and fructose that needs to be broken apart. This process known as glycolysis will break down glucose-producing pyruvate and then it goes into the citric acid cycle to produce ATP. Fructose will break down first, forming fructose 1-phosphate by the enzyme, fructokinase. Aldolase B then will convert the fructose 1-phosphate into DHAP and glyceraldehyde as they enter into the glycolysis pathway. When there is a deficit or a mutation of Aldolase B gene, then hereditary fructose intolerance will occur. Individuals are usually asymptomatic until they ingest sucrose, fructose or sorbitol. When the fructose is ingested, there is a block of the Aldolase B, which will cause an increase of the F1P. This is an autosomal recessive condition from mutation of the Aldolase gene. As of 1991, there have been eight structural defects found in the Aldolase B gene, as documented by Wikipedia. Mutant alleles result in many different mutations; one being base pair substitutes, small deletions, and areas of the splicing regions of the Aldolase B gene. The two by products of F1P can then metabolize and produce glucose, uric acid, lactate and glycogen. Without this process, the body is unable to break down fructose leading to the Cori Cycle. When the...
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...reaction without being used or modified and the thermodynamic properties of the reaction do not change (Hudon-Miller, 2012). 2. (Gresham HS IB Biology, 2007) 3. (Shmoop Editorial Team, 2008) 4. The first two steps of fructose metabolism in the liver is fructose is broken down by fructokinase into fructose -1-phosphate substrate and then Aldolase B converts fructose-1-phostpate into DHAP-glyeraldehyde product. At this step, it can go into glycolysis and make ATP or gluconegenesis to eventually make glycogen (Hudon-Miller, 2012). 5. a. With the lack of aldolase B, there is no longer made product of DHAP-glyerldehyde and therefore, there is no product going into glycolysis to make ATP or product going into gluconegenesis to eventually make glycogen. Fructose is still being broken down to make fructose-1-phospate but it is building up in the liver because there is not enzyme to convert it to DHAP-glyerldehyde. Fructose is no longer being used as energy in the liver (Sanders, 2012). b. Fructose is still being broken down to make fructose-1-phospate but it is building up in the liver because there is not enzyme to convert it to DHAP-glyerldehyde. The phosphate is stuck with fructose and as this is being made, phosphate is being depleted in the cells. The Electron Transport Chain uses free phosphate to make ATP. If there is a lack of phosphate then the Electron Transport chain cannot make ATP. The production of ATP slows down and the...
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...H2 H2 surname first name m.i. surname first name m.i. Name: Yr & Sec: SCORE SCORE Glycolysis For this activity, refer to the figure on next page to complete the table below. Use BLUE INK PEN. Due on March 08 (TTh Classes) and March 09 (MWF and WF classes) Step | Reactant | Product | Enzyme | Type of Reaction | 1 | | | | | 2 | | | | | 3 | | | | | 4 | | | | | 5 | | | | | 6 | | | | | 7 | | | | | 8 | | | | | 9 | | | | | 10 | | | | | Identification: 1. 2. Two steps in glycolysis that use ATP 3. Steps in glycolysis where molecule is split. 4. Two steps in glycolysis that produce ATP. 5. # of NADH produced: _____ 6. # of carbons found in the glucose: _____ 7. # of carbons found in each pyruvate molecule: _____ 8. # of ADP produced: _____ 9. # ATP produced: _____ 10. Net total of ATP produced: _____ 11. Which product of glycolysis is used in liver for synthesis of triglycerides? Glycolysis - occurs in the cytoplasm that converts glucose into two pyruvates * can be aerobic or anaerobic (erythrocytes and exercising skeletal muscles) * Step 1 Step 1 Step 10 Step 10 Step 9 Step 9 Step 8 Step 8 Step 7 Step 7 Step 6 Step 6 Step 5 Step 5 Step 4 Step 4 Step 3 Step 3 Step 2 Step 2 Citric Acid Cycle/Tricarboxylic acid Cycle (TCA)/ Krebs Cycle *...
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... but remains unchanged. Secondly they increase rates without changing the chemical equilibrium between reactants and product. This allows the enzyme to move to the next substrate and the cycle starts over again. Gresham HS IB Biology. (2007) Wolfe,G.(2000) Fructose is almost completely metabolized in the liver. There are two steps of fructose metabolism. The first step to breakdown fructose is to split the substrate into fructose 1 phosphate. This is called fructokinase. The second step of fructose metabolism is that it undergoes hydrolysis of fructose 1 phosphate by aldolase B, which is an enzyme that breaks down into DHAP and glyceraldehyde. These products enter the glycolysis pathway where they are converted to pyruvate. Pyruvate is a necessary molecule for the production of ATP and the citric acid cycle. Aldolase B insufficiency could be a mutation in the ALDOB gene. This deficiency stops fructose from breaking down at the fructose 1phosphate stage. This causes a build up in the liver, thus causing a depletion of phosphate necessary to make adenosine triphosphate. With this deficiency the synthesis of sugar cannot occur. Fructose...
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