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Quantitative Proteomic Study of Myocardial Mitochondria in Urea Transporter B Knockout Mice.
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HADHA(Trifunctional enzyme subunit alpha, mitochondrial) is also named as HADH,78 kDa gastrin-binding protein.It belongs to the enoyl-CoA hydratase/isomerase family in the N-terminal section and the 3-hydroxyacyl-CoA dehydrogenase family in the central section.It harbors the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities.Defects in HADHA are a cause of trifunctional protein deficiency (TFP deficiency) and long-chain 3-hydroxyl-CoA dehydrogenase deficiency (LCHAD deficiency) and maternal acute fatty liver of pregnancy (AFLP).