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TFP1/HADHA Antibody

Rabbit Polyclonal Antibody

     
  • WB - TFP1/HADHA Antibody ABV10552-30
    Western blot analysis of TFP-1 expression with lysate from Jurkat cells (Lane 1,2), 3T3 cells (Lane 3) and rat kidney (Lane 4).
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Product Information
Application
  • Applications Legend:
  • WB=Western Blot
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin-embedded Sections)
  • IHC-F=Immunohistochemistry (Frozen Sections)
  • IF=Immunofluorescence
  • FC=Flow Cytopmetry
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • E=ELISA
  • IP=Immunoprecipitation
  • DB=Dot Blot
  • CHIP=Chromatin Immunoprecipitation
  • FA=Fluorescence Assay
  • IEM=Immunoelectronmicroscopy
  • EIA=Enzyme Immunoassay
WB
Primary Accession P40939
Reactivity Human, Mouse, Rat
Host Rabbit
Clonality Polyclonal
Isotype Rabbit IgG
Calculated MW 83000 Da
Additional Information
Gene ID 3030
Application & Usage Western blotting (0.5-4 µg/ml). However, the optimal concentrations should be determined individually. The antibody recognizes 84 kDa TFP1 from samples of human, mouse and rat origins. Reactivity to other species has not been tested.
Other Names Mitochondrial Trifunctional Protein
Target/Specificity TFP1
Antibody Form Liquid
Appearance Colorless liquid
Formulation 100 µg (0.5 mg/ml) affinity purified rabbit polyclonal antibody in 1X phosphate-buffered saline (PBS) containing 30% glycerol, 0.5% BSA, and 0.01% thimerosal.
Handling The antibody solution should be gently mixed before use.
Reconstitution & Storage -20 °C
Background Descriptions
PrecautionsTFP1/HADHA Antibody is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name HADHA
Synonyms HADH
Function Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway (PubMed:8135828, PubMed:1550553, PubMed:29915090, PubMed:30850536). The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA (PubMed:29915090). Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids (PubMed:30850536). Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA described here carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities while the trifunctional enzyme subunit beta/HADHB bears the 3-ketoacyl-CoA thiolase activity (PubMed:8135828, PubMed:29915090, PubMed:30850536). Independently of the subunit beta, the trifunctional enzyme subunit alpha/HADHA also has a monolysocardiolipin acyltransferase activity (PubMed:23152787). It acylates monolysocardiolipin into cardiolipin, a major mitochondrial membrane phospholipid which plays a key role in apoptosis and supports mitochondrial respiratory chain complexes in the generation of ATP (PubMed:23152787). Allows the acylation of monolysocardiolipin with different acyl-CoA substrates including oleoyl-CoA for which it displays the highest activity (PubMed:23152787).
Cellular Location Mitochondrion. Mitochondrion inner membrane Note=Protein stability and association with mitochondrion inner membrane do not require HADHB.
Research Areas
Citations (0)
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Background

Mitochondrial Trifunctional Protein (TFP) is a multienzyme complex of the beta-oxidation cycle. TFP deficiency is a clinically heterogeneous disorder with phenotypes of different severity. The spectrum of diseases range from severe neonatal/infantile cardiomyopathy and early death to mild chronic progressive sensorimotor poly-neuropathy with episodic rhabdomyolysis. Human TFP is an octomer composed of four alpha-subunits and four beta-subunits. Mutations in either subunits may result in general TFP deficiency with reduced activity of all enzymes.

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Discontinued
Cat# ABV10552-30
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