Glucose is the primary source of energy for cells in the body. Normally, glucose is broken down in cells by aerobic respiration to produce large amounts of ATP. However, individuals suffering from PDCD lack Pyruvate dehydrogenase Complex (PDC) forces the body to break down glucose anaerobically to produce large amounts of Latic acid and 2 ATP instead of just 36 ATP.
Pyruvate dehydrogenase Complex (PDC) is involved in converting pyruvate into Acetyl-CoA in the body after glycolysis in aerobic respiration. Acetyl-CoA is essential to kick start the kerb cycle and eventually electron transport chain in order to produce large amount of ATP for the body. (more on the biochemistry behind PDCD and PDC in another post)
Pyruvate dehydrogenase Complex (PDC) is involved in converting pyruvate into Acetyl-CoA in the body after glycolysis in aerobic respiration. Acetyl-CoA is essential to kick start the kerb cycle and eventually electron transport chain in order to produce large amount of ATP for the body. (more on the biochemistry behind PDCD and PDC in another post)
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| Overview of the glucose metabolic process in cells of a person without Pyruvate Dehydrogenase Complex Deficiency (Taken from Caveats when considering ketogenic diets for the treatment of pyruvate dehydrogenase complex deficiency written by Timothy A Weber, BS, M.Rita Antognetti, MS, RD and Peter W, Stacpoole, Phd, MD) |
Those who suffer from PDCD are unable to convert pyruvate to Acetyl-CoA through oxidative decarboxylation.
Puruvate -----------x-------------> Acetyl-CoA
Pyruvate Dehydrogenase Complex
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| Taken from page 87 of Medical Biochemistry written by Dr Zhu CongJu Ngee Ann Polytechnic School of Life Science and Chemical Technology |
As seen in the image above, shows that weather glucose is aerobically or anaerobically broken down, glycolysis occurs to produce pyruvate.
The severity of Pyruvate dehydrogenase complex deficiency can be categorised into type A, B and C.
Type A: Begins in infancy. They have mild lactic acidosis resulting in developmental delay (e.g learning to sit up or lift their neck) vomiting, muscle weakness, abdominal pain and tiredness.
Type B: Begins shortly after birth. Serious lactic acidosis and Hyperammonemia is also a common symptom. THisresults in liver failure, abnormal eye movements, weak muscle tone, coma and seizures. These children usually only manage to survive for several months.
Type C: Benign form of the disease. The elevation of latic acid concentration in their blood is mild and only occurs periodically. Such individuals manage to lead a normal life.
ATP: Adenosine Triphosphate- The only form of energy used by the body
Lactic acidosis: The build up of lactic acid in the blood which results in PH of the blood to fall to below PH7.35-7.45 (e.g PH5)
Hyperammonemia: excess ammonia in the blood (although hyperammonemia is not caused by PDCD, is has been observed people who suffer from PDCD often have hyperammonemia coupled with PDCD. The cause of hyperammonemia in those with PDCD is still unknown)
Why is ATP important? What do you mean by it being the only form of energy? Don't food give us energy too?
ReplyDeleteATP is important because it is a form of energy that the body can use. Energy is important so that we can function normally and carry on with our daily activities. It being the only form of energy means that the body recognises it and can use it. For example, plants can use light energy but humans cannot, else our cells will die. Therefore, our form of energy is by using ATP. Yes, you are right. Food gives us energy. However, food is a more complex substance and it will break down to give us ATP in metabolic pathways in the body. Hope that answers your question. Thanks for the question :)
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