Hereditary Fructose Intolerance Research Paper
Microbiology
Enzymology and Catalytic Metabolism
Hereditary Fructose Intolerance (HFI)
Hereditary Fructose Intolerance (HFI) is a genetic condition people are born with, usually without previous family history. Individuals with this condition have difficulty metabolizing 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-1-phosphate aldolase) which in turn causes the build-up of fructose-1-phosphate . The overabundance of fructose-1-phosphate prevents glycogen breakdown and ultimately the …show more content…
Fructose is phosphorylated by the enzyme hexokinase to form fructose-6-phosphate, an intermediate of glycolysis.
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 …show more content…
CoQ10 is the electron carrier between the enzyme complexes required to complete this process of ATP production in every cell within the body to produce energy.
References
Bayir & Kagan. (2008). Critical Care. 12 (206). doi:10.1186/cc6779
Biology 1. (2012). Rockhill. SC. Retrieved from http://mrmackrh.weebly.com/
HealthHype.com. (2013). Fructose Enzyme Deficiency Causes, Symptoms and Treatment. Retrieved from http://www.healthhype.com/fructose-enzyme-deficiency-causes-symptoms-and-treatment.html
Medscape Reference. (2013). Drugs Diseases and Procedures. Aldolase. Retreived from http://emedicine.medscape.com/article/2087158-overview#a30 New World Encyclopedia. (2012). Fructose. Retrieved from http://www.newworldencyclopedia.org/p/index.php?title=Fructose&oldid=964792. Teachers Instructional Graphics Educational Resource (TIGER). (2008). North Carolina School of Science and Mathematics. Durham. NC. Retrieved from http://www.dlt.ncssm.edu/stem/
The Cori Cycle. 20 (2). Interactive Concepts in Biochemistry. Retrieved from
Enzymology and Catalytic Metabolism
Hereditary Fructose Intolerance (HFI)
Hereditary Fructose Intolerance (HFI) is a genetic condition people are born with, usually without previous family history. Individuals with this condition have difficulty metabolizing 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-1-phosphate aldolase) which in turn causes the build-up of fructose-1-phosphate . The overabundance of fructose-1-phosphate prevents glycogen breakdown and ultimately the …show more content…
Fructose is phosphorylated by the enzyme hexokinase to form fructose-6-phosphate, an intermediate of glycolysis.
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 …show more content…
CoQ10 is the electron carrier between the enzyme complexes required to complete this process of ATP production in every cell within the body to produce energy.
References
Bayir & Kagan. (2008). Critical Care. 12 (206). doi:10.1186/cc6779
Biology 1. (2012). Rockhill. SC. Retrieved from http://mrmackrh.weebly.com/
HealthHype.com. (2013). Fructose Enzyme Deficiency Causes, Symptoms and Treatment. Retrieved from http://www.healthhype.com/fructose-enzyme-deficiency-causes-symptoms-and-treatment.html
Medscape Reference. (2013). Drugs Diseases and Procedures. Aldolase. Retreived from http://emedicine.medscape.com/article/2087158-overview#a30 New World Encyclopedia. (2012). Fructose. Retrieved from http://www.newworldencyclopedia.org/p/index.php?title=Fructose&oldid=964792. Teachers Instructional Graphics Educational Resource (TIGER). (2008). North Carolina School of Science and Mathematics. Durham. NC. Retrieved from http://www.dlt.ncssm.edu/stem/
The Cori Cycle. 20 (2). Interactive Concepts in Biochemistry. Retrieved from