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Nan
Forum Senior Topics: 12 Posts: 159 |
for the tx of PDH deficiency, could you explain the reason of high fat content or increasing lysine and leucine? Thanks in advance.
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Palaniappan
| Forum Senior Topics: 30 Posts: 148
I think in PDH deficiency..glycolysis cannot occur to produce acetyl coa thereby -->no energy through glycolysis. When you give fat-->beta oxidation--->acetyl coa-->tca-->energy Leucune,isoleucine-->direct catabolism-->acetyl coa-->energy. This might be a possible explanation.Please correct me if I am wrong.
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alb123
| Forum Junior Topics: 2 Posts: 55
You are right. good explanation.
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Nan
| Forum Senior Topics: 12 Posts: 159
thanks for the explanation. In addition, Lysine and Leucine are kegtogenic. both can be used for ketone boides.
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bd2005
| Forum Newbie Topics: 7 Posts: 24
I think the mechanism is like this: PDH deficiency=> Decrease in Acetyle coA => Decrease in TCA cycle. Now guys, you can recall that Isoleucine, leucine and Valine are the three branched chain keto acids that are metablised by branched chain keto acid dehydrogenase enzyme deficiency of which causes Maple syrup urine disease. This enzyme forms acetyle coa and propeonyl coA from those amino acids.Ultimately these acetyle coa and propeonyl coA enter TCA cycle. In PDH deficiency, that is not possible. Again odd carbon fatty acids are metabolised to propeonyl coA =>methylmalonyl CoA=> Succinyl coA => TCA cycle. Again due to the suppression of TCA cycle this can not happen. For these, we find high level of Isoleucine, Leucine, Valine and Odd carbon fatty acids in blood in a apatient with PDH deficiency Cheers bd200004@yahoo.com
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