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HADHA (F9M4C) Rabbit Monoclonal Antibody #50257

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  • WB

    Product Specifications

    REACTIVITY H
    SENSITIVITY Endogenous
    MW (kDa) 80
    Source/Isotype Rabbit IgG
    Application Key:
    • WB-Western Blotting 
    Species Cross-Reactivity Key:
    • H-Human 

    Product Information

    Product Usage Information

    Application Dilution
    Western Blotting 1:1000
    Simple Western™ 1:50 - 1:250

    Storage

    Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at –20°C. Do not aliquot the antibody.

    Protocol

    Specificity / Sensitivity

    HADHA (F9M4C) Rabbit Monoclonal Antibody recognizes endogenous levels of total HADHA protein. This antibody does not cross-react with HADHB protein.

    Species Reactivity:

    Human

    Source / Purification

    Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Val168 of human HADHA protein.

    Background

    Mitochondrial trifunctional enzyme, also known as mitochondrial trifunctional protein (MTP), catalyzes three reactions in the fatty acid β-oxidation pathway. It has two different types of subunits: α/HADHA and β/HADHB. HADHA possesses the 2,3-enoyl-CoA hydratase (ECH) and 3-hydroxyacyl-CoA dehydrogenase (HACD) activities, while HADHB has 3-ketoacyl-CoA thiolase activity (1-3). Studies show that HADHA stimulates ketone body production through fatty acid β-oxidation. β-hydroxybutyrate, one of the ketone bodies, inhibits gluconeogenesis (1). Furthermore, studies have identified mutations in HADHB that cause MTP deficiency, an autosomal recessive disorder severely restricting long-chain fatty acid oxidation (4-6).

    Alternate Names

    3-ketoacyl-Coenzyme A (CoA) thiolase, alpha subunit; 3-oxoacyl-CoA thiolase; 78 kDa gastrin-binding protein; ECHA; gastrin-binding protein; GBP; HADH; HADHA; hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha; hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit; hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit; LCEH; LCHAD; Long chain 3-hydroxyacyl-CoA dehydrogenase; long-chain 2-enoyl-CoA hydratase; Long-chain enoyl-CoA hydratase; long-chain-3-hydroxyacyl-CoA dehydrogenase; MGC1728; mitochondrial long-chain 2-enoyl-Coenzyme A (CoA) hydratase, alpha subunit; mitochondrial long-chain L-3-hydroxyacyl-Coenzyme A (CoA) dehydrogenase, alpha subunit; mitochondrial trifunctional enzyme, alpha subunit; mitochondrial trifunctional protein, alpha subunit; Monolysocardiolipin acyltransferase; MTPA; TP-alpha; Trifunctional enzyme subunit alpha, mitochondrial

    For Research Use Only. Not for Use in Diagnostic Procedures.
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