Overview

mitochondrial trifunctional protein


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(TFP)

A mitochondrial multienzyme complex that catalyses the last three steps in the long-chain fatty acid β-oxidation pathway. There are four hydroxyacyl-CoA dehydrogenase-α (HADHA, 79 kDa) and four hydroxyacyl-CoA dehydrogenase-β (HADHB, 48 kDa) subunits. TFP deficiency leads to a wide clinical spectrum of disorders.

Subjects: Medicine and Health.


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