PDH Complex Def: The B1 Trap behind Wernicke-Korsakoff Syndrome | MBBS USMLE

[00:00:01]chemical aspects of pdh complex if we talk about the clinical aspects few points that are very important to remember number one whenever there is the dietary deficiency of this p1 whenever there is the type 3 deficiency of this b1 then the cells will be deficient with this TPP when the cells will be deficient with TPP there will no conversion of pyruvate into acetyl CoA and the body will suffer from the deficiency of energy and it can cause serious complication that will that I will explain at the end chicken this the fish the person's are the people who are alcoholic because alcoholism it causes the deficiency of this b11 this when someone is alcoholic there will be the deficiency of b1 and when there is a deficiency of b1 there will be that deficiency of TPP there will be no TPP formation and when there is no TPP formation there will be no conversion of pyruvate into acetyl-coa and no energy in the cells and a third one which is the very important third one is the inherited efficiency of this pyruvate dehydrogenase whenever the person is suffering from then the at the genetic level suffering from the deficiency of this pyruvate dehydrogenase enzyme because of some mutation or something else there will be no formation of this acetyl CoA and the body will suffer from the deficiency of energy and the third clinical most important point is the arsenic poisoning arsenic poisoning Julia arsenic is present in two forms trivalent as well as pentavalent the pentavalent contaminant form of this arsenic it will affect the glycolytic pathway well the trivalent form of arsenic it will compete with this lipoic acid and other that this lipoic acid will not be able to continue this pathway our this lipoic acid when this arsenic trivalent form of this arsenic is going to replace its lipoic acid that is going to compete with the flipper equation then there will be no formation of this a seat I'll lick pomide and other than the reaction will not proceed in forward direction R it will not yield the two molecules of NADH T the NADH to and when there will be no formation of NADH to then there will be no energy now with these technical deficiencies either it is the dietary deficiency of VO on other it is the alcoholic deficiency of deficiency of b1 because of alcoholism r it is the arsenic poisoning it can cause serious complications and the most importantly this arsenic causing it is not going to affect so much on the brain this this b1 it has serious effects from the brain because our CNS it mostly will rise on the energy CNS it consistently needs energy and for this to fulfill the demands of this there must be Arabic respiration or Arabic process and when our there is the deficiency of this b1 thymine b1 or thiamine deficiency then there will be no formation of NADH 2 and acetyl co and when there is no formation of NADH 2 and acetyl co a the TCA cycle will not operate and CN n-- s it will become deficient with the energy when CNS will become deficient with the energy it can cause the serious complications like it can damage the brain you have listened you have I think known of a disease warnock koskoff disease that disease is because of this b1 deficiency and when there is a Beeman deficiency the energy pathway is disturbed and that will affect the C and s and the there will be the encephalopathy in Warnock koskoff disease which I have told you that which is with the because of the deficiency of b1 and what is the row of this b1 this b1 is going to play a very important role in this pdh complex through the TPP as well as neuropathy neuropathies also a consequence off we can say that b1 deficiency in few its experiments into a few scientists they have proved that b1 has important role in the optic neuropathy in the cause of in causing the optic neuropathy so this b1 is very important and as well as the drive in it it can also disturb the pdh complex so these are the clean these were the clinical aspects that you must know that you must be well aware of it once again I am going to repeat that whenever maybe the title deficiency of b1 or the alcoholic deficiency because of alcoholism the deficiency of B walk this is going to affect pdh complex how it is going to affect the pdh complex it is going to target especially the CNS by disturbing this pdh complex how this b1 normally it is converted into the TPP which is the thymine pyrophosphate and this time in pyrophosphate is an important coenzyme of the e1 of pdh complex when there is no b1 there will be no TPP when there is no TPP even will not work when this will not work there will be no formation of acetylcholine ATP and when acetyl co a is not formed the TCA cycle will not operate in the brain and brain is very we can say some strip fourth energy and when this brain is going to be deficient with energy then that will damage the CNS so this was a clinical significance of this pdh complex