DISORDERS OF METABOLISM
By Wieslaw Faliszewski
Our cells are using various types of substances in order to perform their functions. They also use them as a source of energy to perform all the required tasks. Some of those compounds come from the outside in the form of consumed food; others are synthesized in our body.
Majority of those compounds have to be broken into simpler parts that can be used in different metabolic processes. The problem is, however, that most of the complex molecules are quite stable and do not disintegrate easily on their own. They will require a very long time or high amount of energy to disintegrate into useful particles. There are, however, substances that can help in the process of breaking …show more content…
The cycle starts when the enzymes strip off the CoA portion from acetyl-CoA and combine two-carbon acetyl group with a four-
carbon oxaloacetate to form six-carbon citric acid (hence the other name for the cycle is citric acid cycle). The process of creating citric acid is facilitated by enzyme called citrate synthase.
Krebs cycle in its simple form is the process of modifying citric acid in several steps till the molecule becomes the four-carbon oxaloacetate again. More precisely, steps of Krebs cycle are the progressive oxidation of citric acid. Oxidation is a chemical reaction where electrons and protons are removed form molecule. In the case of TCA cycle, electrons and protons are switched onto two electron carriers: FADH2 and NADH, which can take part in the electron transport chain (Campbell & Farrell, 2010).
Steps of citric acid cycle are completely dependent on above mentioned enzymes that reside in the matrix of mitochondria. Every step is accomplished by its own enzyme that use the product of previous enzyme as a substrate. Therefore, if any of those enzymes will …show more content…
We can hypothesize, for example, that if malate dehydrogenase will cease to do its job in Krebs cycle, two major problems will occur, as a result. First, malate dehydrogenase is the one of the enzymes that facilitates electron transport to NAD+ and creating NADH. Therefore, the final output of electron carrier NADH will be reduced to eight from ten per molecule of glucose, rendering the whole process less efficient.
Second, the product of malate dehydrogenase is oxaloacetate, the same molecule that is a substrate in the process of synthesizing citric acid. Without malate dehydrogenase, we will suffer severe oxaloacetate deficiency. Unless it will be supplied by other sources, the whole Krebs cycle will halt, disastrously reducing our energy production. This in turn could lead to so called
Chronic Fatigue Syndrome or even death.
Other hypothetical, enzyme related problem, can exist in the electron transfer chain when coenzyme Q10 (CoQ10) is not fulfilling its function. CoQ10 is one of several enzymes that reside inside inner mitochondrial membrane. Its unique and important function is a collection of electrons from complexes I and II, and transferring them to the complex III. In other