Metabolism Hereditary Fructose Intolerance (WHIFF) 0 Hereditary Fructose Intolerance (WHIFF) is a genetic condition people are born with, usually without previous family history. Individuals with this condition have difficulty metabolize fructose and/or foods containing fructose. The individuals liver and kidneys attempt to use this sugar for energy and due to the incomplete breakdown of fructose, toxic byproducts are produced which eventually leads to serious illness. The genetic condition causes an enzyme deficiency (fructose-I-phosphate elodeas) which in turn causes the build-up of fructose-I-phosphate . The overabundance of fructose-I-phosphate prevents glycogen breakdown and ultimately the synthesis of glucose for the body to use as energy source. With the inhibition of glucose synthesis after ingesting fructose, the individual experiences sever hypoglycemia The symptoms of WHIFF area as follows: Dislike of fruits, vegetables, candies, and baked goods. Love of dextrose-based candies.
Preference of beverages such as milk, water, unsweetened tea, and unsweetened coffee. The feeling of nausea, queasy stomach, shaky, and/or foggy shortly after insuring fructose or sucrose. Experiencing kidney pain, hypoglycemia, and/or weakness after consuming fructose or sucrose (may be a few hours to days). The individual may eat large quantities of “safe” foods, e. G. , dairy products, pasta, potato chips and rice, after ingesting fructose. 0 If fructose is ingested, other symptoms such as vomiting, hypoglycemia, Jaundice, hemorrhage, heptagonal, hyperglycemia and eventually kidney failure will follow. Treatment of WHIFF is usually difficult and requires a strict fructose free diet and the exclusion of foods containing fructose, sucrose or servitor. Errantly done through a strict fructose free diet. Hereditary Fructose Intolerance (WHIFF), cont’d 0 A deficiency of the enzyme elodeas B cay be responsible for WHIFF due to the following; 0 Inhibition or a reduction in the breakdown of fructose I-phosphate by elodeas B inhibits another enzyme known as frustrations. 0 Free fructose may accumulate in the bloodstream, liver and kidneys.
The high fructose levels in the body affect other necessary cellular reactions and an increase in uric acid levels in the blood may be noted. 0 The excretion of lactic acid by the kidneys may be affected u to the kidneys attempt to excrete the increased level of uric acid which in turn may cause lactic acidosis. 0 Elodeas B is necessary for the production of glucose through glutinousness , so a decreased level of elodeas B may cause a failure to regulate the glucose available in the body and hypoglycemia usually results.
Enzymatic Involvement in Breakdown of Fructose 0 The breakdown to truces may take one to two pathways and depends on the enzymes used during the phosphorescently. The affinity of frustrations for fructose is higher than that of huskiness. 0 The liver is the predominant pathway for fructose reawaken 0 The liver breaks down glucose and fructose differently. There are three steps involved in the fructose-I-phosphate pathway, which is performed by the liver due to its high concentration of frustrations relative to huskiness: 0 Fructose is phosphorescently by the enzyme frustrations to fructose-I-phosphate. Adipose and skeletal muscle tissue also play a part in fructose breakdown but on a much smaller scale that the liver. 0 The breakdown of fructose in these tissues is similar to the catabolic of glucose. 0 Fructose is phosphorescently by the enzyme huskiness to form fructose-6-phosphate, an intermediate of glycoside. Diagrams of enzymatic activity As depicted in the diagram above, an enzyme acts a catalyst to lower the activation energy of a reaction. The enzyme’s conformation is such that the substrates easily bind to the enzyme and with the addition of sufficient energy to break bonds, the products are released.
As you can see, the enzyme is completely unaltered in the process and can perform the same reaction over and over without a loss of effectiveness. Effect of Enzymes on Activation Energy As depicted in the graph to the left, you can see the decrease in activation energy required when enzymes are used. The reaction in which an enzyme acts as a catalyst (red line) uses considerably less energy expenditure to start the reaction (activation energy). Without the use of a catalyst (enzyme) the activation energy is considerably greater. The amount of energy released during both reactions is the same.
By using an enzyme to decrease the activation energy required, the net amount of energy recovered is greater. Action of Elodeas B Elodeas B is an enzyme which plays a key role in converting sugar into energy. This process occurs mostly in the liver, kidney, and mucosa of the small intestine. Elodeas B plays a particularly important role in fructose metabolism, specifically splitting frustrations to form fructose I-phosphate (F,P) then elodeas B catalysts Fl P into clearheadedly and ADAPT. After clearheadedly is phosphorescently by trios kinas to form APP, both of these products may then be made into glucose or private.
Elodeas has three subgroups where subgroup B can work in both glycoside and glossiness’s by the liver and kidney. Subgroups A and C are primarily used in glycoside by the muscles, erythrocytes and the brain. What if the Coir Cycle Occurred Within a Single Cell Since the cycle causes a loss of TAP, the cell would naturally loose energy. The glycoside part of the Coir cycle produces 2 TAP molecules at a cost of using 6 TAP molecules. Each turn of the cycle requires a net use of 4 TAP molecules. As a result, the cycle cannot be sustained indefinitely. Within one cell.
The consumption of TAP molecules mandates the Coir cycle sin TTT the metabolic burden trot inside one individual cell to the liver. Instead of accumulating inside the individual cell, the anaerobic production of lactate is taken up by the liver where the other half of the Coir yecch can begin. In the liver, glutinousness occurs. The glucose is then supplied to the individual cells through the bloodstream and can be fed back into the glycoside reactions in the cell. Hypothetical Defect in the Citric Acid Cycle A defect which may affect the Citric acid cycle would be the genetic malformation in the structure of one of the required enzymes I. . , citrate dehydrogenate. This would prevent the cycle from completing the entire process at the point where the necessary enzyme would be lacking and severely inhibit the production of TAP and he potential build up of substances to toxic levels. ; A deficiency in vitamins in an individual’s nutritional intake could affect the Citric acid cycle as well. ; A riboflavin deficiency would affect the citric acid cycle due to the fact the flaming nucleotides are required for the synthesis of FAD. If FAD could not be synthesized, the enzyme succinctness dehydrogenate would not work correctly and the entire cycle would be negatively affected.