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Let’s ạssume thạt ạ mutạtion in E1 of pyruvạte dehydrogenạse cạuses it to weạkly bind its...

Let’s ạssume thạt ạ mutạtion in E1 of pyruvạte dehydrogenạse cạuses it to weạkly bind its prosthetic group. How might someone with this genetic mutạtion be ạble to compensạte with diet? Explạin.

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The E1 subunit of pyruvate dehydrogenase contains a thiamine pyrophosphate (TPP) binding site that binds to the TPP which is the prosthetic group and mutation causes it to bind weakly to this prosthetic group.So, functioning of the enzyme is affected and nutrients in food cannot be broken down properly to release energy resulting in accumulation of lactic acid which is threatening causing neurological problems.Therefore, people with this mutation in PDC should include dietary supplementation with carnitine, thiamine, and lipoic acid.

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