Overview

Description

  • Nature
    Synthetic

Associated products

Specifications

Our Abpromise guarantee covers the use of ab155748 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    Blocking - Blocking peptide for Anti-HADHA antibody (ab54477)

  • Form
    Liquid

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

    pH: 7.20
    Preservative: 0.02% Thimerosal (merthiolate)
    Constituents: PBS, 50% Glycerol, 1% BSA

General Info

  • Alternative names
    • 3 ketoacyl Coenzyme A (CoA) thiolase alpha subunit
    • 3 oxoacyl CoA thiolase
    • 78 kDa gastrin binding protein
    • 78 kDa gastrin-binding protein
    • ECHA
    • ECHA_HUMAN
    • GBP
    • HADH
    • HADHA
    • 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
    • Mitochondrial long chain 2 enoyl Coenzyme A (CoA) hydratase alpha subunit
    • Mitochondrial long chain L 3 hydroxyacyl Coenzyme A dehydrogenase alpha subunit
    • Mitochondrial trifunctional enzyme alpha subunit
    • Mitochondrial trifunctional protein alpha subunit
    • MTPA
    • Thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit
    • TP ALPHA
    • TP-alpha
    • Trifunctional enzyme subunit alpha mitochondrial precursor
    see all
  • Function
    Bifunctional subunit.
  • Pathway
    Lipid metabolism; fatty acid beta-oxidation.
  • Involvement in disease
    Defects in HADHA are a cause of trifunctional protein deficiency (TFP deficiency) [MIM:609015]. The clinical manifestations are very variable and include hypoglycemia, cardiomyopathy and sudden death. Phenotypes with mainly hepatic and neuromyopathic involvement can also be distinguished. Biochemically, TFP deficiency is defined by the loss of all enzyme activities of the TFP complex.
    Defects in HADHA are the cause of long-chain 3-hydroxyl-CoA dehydrogenase deficiency (LCHAD deficiency) [MIM:609016]. The clinical features are very similar to TFP deficiency. Biochemically, LCHAD deficiency is characterized by reduced long-chain 3-hydroxyl-CoA dehydrogenase activity, while the other enzyme activities of the TFP complex are normal or only slightly reduced.
    Defects in HADHA are a cause of maternal acute fatty liver of pregnancy (AFLP) [MIM:609016]. AFLP is a severe maternal illness occurring during pregnancies with affected fetuses. This disease is associated with LCHAD deficiency and characterized by sudden unexplained infant death or hypoglycemia and abnormal liver enzymes (Reye-like syndrome).
  • Sequence similarities
    In the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase family.
    In the central section; belongs to the 3-hydroxyacyl-CoA dehydrogenase family.
  • Cellular localization
    Mitochondrion.
  • Information by UniProt

References

ab155748 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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