Mitochondrial trifunctional protein (TFP) (HADHA / HADHB)

UniProt Number: P40939 & P55084
Alternate Names: Trifunctional enzyme subunit alpha and Trifunctional enzyme subunit beta, Long-chain enoyl-CoA hydratase, Long chain 3-hydroxyacyl-CoA dehydrogenase, 3-ketoacyl-CoA thiolase
Structure and Function: 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.
Disease Associations: 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.

Monoclonal Antibodies
Cat. No. Name Reactivity Apps. Amount Price  
MS702 TFP subunit alpha (HADHA) antibody human WB, ICC, IP, ICE, FLOW 100 µg $325.00
MS733 TFP subunit beta (HADHB) antibody human WB, ICC, IP, ICE, FLOW 100 µg $325.00
MS734 TFP subunit alpha and beta (HADHA / HADHB) IHC antibody human, bovine, rat ICC, IHC, IP, FLOW 100 µg $325.00

Protein Quantity Assays
Cat. No. Name Reactivity Amount Price  
MSFX-11 TFP subunit alpha and beta (HADHA / HADHB) FlexPlex™ Module human 4 strips (32 tests) $125.00

Multiplexing Arrays
Cat. No. Name Reactivity Amount Price  
MSX32 MetaPath™ Fatty Acid Oxdiation 4-Plex Dipstick Array human 30 tests $545.00
90 tests $995.00

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Related Pathways:

Acetyl-CoA Metabolism: Key Nodes

Fatty Acid Oxidation (Mitochondrial)

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