Show Video TranscriptHello students. Today's question is about the genetic disease. So that is the pyro. Wait, the hydrogen is mutation or deficiency. So pyro waited the hydrogen is enjoyment that the hydrogen is enjoying deficiency means that it is not working. It is not working. So if it is not working, there is another consequences. So that is a build up of the lactic acid build up of the lactic acid. And following are the different options that we have to explain why Pyro wait, if a pirouetted, the hydrogen is enjoyment, is not working the fire away to the hydrogen ease in jail time is not working, not working. Why there is a build up of the lactic acid how lactic acid is building up. So following are the options. Let's have a look at them. So first taser cell uses lactic acid to shunned electrons from the pirate bay to electron transport chain in mitochondria? No, this is incorrect. Next to sell uses glycol icis. No. Then cell cannot transport pirouette to mitochondria in absence of pirouetting hydrogen, its activities so it is broken down to electric as when an internal note. So the remaining option is the correct answer. Remaining option is the correct answer, which indicates that cell undergo fermentation because pirouette cannot be the metabolized to proceed to the crab cycle. So what happened? We know that we have, we have glycol is season. We have glide policy. So what is glide policy. So that is the conversion of glucose glucose to pirouette two x 2 weeks. But after this, the spiral, it is a converted to acetyl. Coa and it will go to the it will go to the crab cycle Krebs cycle and then the key chain happened electron transport chain. So this is a crucial step that we're talking about here is stelco it this is not happening. This is not happening. Why? Because piru waiter? Because pirou waited the hydrogen ist pyro wait, the hydrogen is's not here. The hydrogen is which actually convert the pirouette pirouette up to us. It'll kuwait. So this is not here, this is not here. So what happened instead is the spiral, it undergoes fermentation. It undergoes fermentation because pirouette is not being converted to a city. So there is increasing concentration of pirouette increase in fire a bit. So something should happen off the spiral bit. So instead the spiral bit will be converted to lactic acid. So all the pyro, wait, that is not being converted to acetyl coa is so that is being converted to lactic acid that we call the electric acidosis sc doses, which is our fatal conditions. So I hope you understood it well, why? This is the correct answer. Thank you Which of the following observations provides the best evidence that acetylWhich of the following observations provides the best evidence that acetyl-CoA negatively regulates pyruvate dehydrogenase activity? presence of a higher concentration of acetyl-CoA.
Where is pyruvate dehydrogenase most likely active?PDC is located in the mitochondrial matrix space, and is responsible for irreversibly converting pyruvate into acetyl CoA, the primary fuel of the citric acid cycle (CAC). Reactions of the CAC and fatty acid oxidation are performed in the mitochondrial matrix.
What happens if pyruvate dehydrogenase is inhibited?Pyruvate dehydrogenase kinase 4 (PDK4) is a regulator of PDH, as it inhibits PDH activity, which in turn will increase the influx of acetyl-coA from beta-oxidation into the TCA cycle, thereby leading to enhanced FA oxidation and slowing of glycolysis or glycolytic intermediates to alternative metabolic pathways.
What does pyruvate dehydrogenase do?The pyruvate dehydrogenase complex converts a molecule called pyruvate, which is formed from the breakdown of carbohydrates, into another molecule called acetyl-CoA. This conversion is essential to begin the series of chemical reactions that produce energy for cells.
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Which of the following best explains the buildup of lactic acid in individuals with the mutation?
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