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Proteins > Mitochondrial trifunctional protein (TFP) (HADHA / HADHB)
Mitochondrial trifunctional protein (TFP) (HADHA / HADHB)
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UniProt Number:
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P40939 & P55084
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Alternate Names:
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Trifunctional enzyme subunit alpha and Trifunctional enzyme subunit beta, Long-chain enoyl-CoA hydratase, Long chain 3-hydroxyacyl-CoA dehydrogenase, 3-ketoacyl-CoA thiolase
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Structure and Function:
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TFP is part of the mitochondrial fatty acid beta-oxidation pathway. It is located in the inner mitochondrial membrane and it catalyzes three out of the four steps in the beta-oxidation cycle with a specific affinity for long chain substrates. TFP is hetero-octamer composed of four alpha and four beta subunits. The alpha subunit is a 79 kDa enzyme which has two enzymatic functions, the hydration of enoyl-CoA and the dehydrogenation of 3-hydroxyacyl CoA. The beta subunit, a 47 kDa enzyme, performs the third function - the thiolytic cleavage of 3-ketoacyl-CoA substrates.
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Disease Associations:
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Defects in HADHA / HADHB are a cause of trifunctional protein deficiency (TFP deficiency) [MIM:609015]. Defects in HADHA are the cause of long-chain 3-hydroxyl-CoA dehydrogenase deficiency (LCHAD deficiency) [MIM:609016]. Defects in HADHA are also a cause of maternal acute fatty liver of pregnancy (AFLP) [MIM:609016]. AFLP is a severe maternal illness occurring during pregnancies with affected fetuses.
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Monoclonal Antibodies
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Cat. No.
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Name
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Reactivity
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Apps.
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Amount
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Price
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MS702
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TFP subunit alpha (HADHA) antibody
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human
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WB, ICC, IP, ICE, FLOW
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100 µg
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$325.00
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MS733
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TFP subunit beta (HADHB) antibody
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human
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WB, ICC, IP, ICE, FLOW
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100 µg
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$325.00
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MS734
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TFP subunit alpha and beta (HADHA / HADHB) IHC antibody
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human, bovine, rat
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ICC, IHC, IP, FLOW
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100 µg
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$325.00
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Protein Quantity Assays
Multiplexing Arrays
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Related Pathways:
Acetyl-CoA Metabolism: Key Nodes
Fatty Acid Oxidation (Mitochondrial)
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