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Overview

  • Product nameHADHA peptideSee all HADHA proteins and peptides ...
  • Protein descriptionSynthetic peptide of Human HADHA.(Note: the amino acid sequence is proprietary)This peptide was used as an immunogen for ab93207 - HADHA antibody.
  • Molecular weight83kDa
  • Protein length15 amino acids

    • Properties

  • Purity70 - 90% by HPLC
  • FormLiquid
  • Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
  • Storage bufferInformation available upon request.
  • Concentration information loading...
  • Research Areas

    • Applications

  • Application notesThis peptide can be used with studies using ab93207.

    • Protein info

    • Alternative names
        3 ketoacyl Coenzyme A (CoA) thiolase alpha subunit3 oxoacyl CoA thiolase78 kDa gastrin binding protein
        78 kDa gastrin-binding proteinECHA_HUMANGBPHADHHADHAHydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunitLCHADLong chain 3-hydroxyacyl-CoA dehydrogenaseMitochondrial long chain 2 enoyl Coenzyme A (CoA) hydratase alpha subunitMitochondrial long chain L 3 hydroxyacyl Coenzyme A dehydrogenase alpha subunitMitochondrial trifunctional enzyme alpha subunitMitochondrial trifunctional protein alpha subunitMTPAThiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunitTP ALPHATP-alphaTrifunctional enzyme subunit alpha mitochondrial precursor
      see all
  • FunctionBifunctional subunit.
  • PathwayLipid metabolism; fatty acid beta-oxidation.
  • Involvement in diseaseDefects 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 similaritiesIn 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 localizationMitochondrion.

    • Target information above from: UniProt accession P40939 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt

    References for HADHA peptide (ab105627)

    ab105627 has not yet been referenced specifically in any publications.

    Publishing research using ab105627 ? Please let us know so that we can cite the reference in this datasheet

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