Description

  • Product name

    Recombinant Human HADHB protein
    See all HADHB proteins and peptides
  • Expression system

    Wheat germ
  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      MTILTYPFKNLPTASKWALRFSIRPLSCSSQLRAAPAVQTKTKKTLAKPN IRNVVVVDGVRTPFLLSGTSYKDLMPHDLARAALTGLLHRTSVPKEVVDY IIFGTVIQEVKTSNVAREAALGAGFSDKTPAHTVTMACISANQAMTTGVG LIASGQCDVIVAGGVELMSDVPIRHSRKMRKLMLDLNKAKSMGQRLSLIS KFRFNFLAPELPAVSEFSTSETMGHSADRLAAAFAVSRLEQDEYALRSHS LAKKAQDEGLLSDVVPFKVPGKDTVTKDNGIRPSSLEQMAKLKPAFIKPY GTVTAANSSFLTDGASAMLIMAEEKALAMGYKPKAYLRDFMYVSQDPKDQ LLLGPTYATPKVLEKAGLTMNDIDAFEFHEAFSGQILANFKAMDSDWFAE NYMGRKTKVGLPPLEKFNNWGGSLSLGHPFGATGCRLVMAAANRLRKEGG QYGLVAACAAGGQGHAMIVEAYPK
    • Amino acids

      1 to 474
    • Tags

      GST tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab158632 in the following tested applications.

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

  • Applications

    ELISA

    Western blot

  • Form

    Liquid
  • Additional notes

    Protein concentration is above or equal to 0.05 mg/ml.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names

    • 2 enoyl Coenzyme A (CoA) hydratase beta subunit
    • 3 ketoacyl Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein beta subunit
    • 3 ketoacyl Coenzyme A thiolase
    • 3 ketoacyl Coenzyme A thiolase of mitochondrial trifunctional protein beta subunit
    • 3-ketoacyl-CoA thiolase
    • Acetyl CoA acyltransferase
    • Acetyl-CoA acyltransferase
    • Beta ketothiolase
    • Beta-ketothiolase
    • ECHB
    • ECHB_HUMAN
    • HADH
    • Hadhb
    • Hydroxyacyl CoA dehydrogenase/3 ketoacyl CoA thiolase/enoyl CoA hydratase (trifunctional protein), beta subunit
    • Hydroxyacyl Coenzyme A (CoA) dehydrogenase beta subunit
    • Hydroxyacyl Coenzyme A dehydrogenase
    • Hydroxyacyl Coenzyme A dehydrogenase beta subunit
    • Hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein) beta subunit
    • MGC87480
    • Mitochondrial trifunctional enzyme beta subunit
    • Mitochondrial trifunctional protein beta subunit
    • MSTP 029
    • MSTP029
    • MTPB
    • TP beta
    • TP-beta
    • TPbeta
    • Trifunctional enzyme subunit beta
    • Trifunctional enzyme subunit beta mitochondrial
    • Trifunctional protein
    see all
  • Pathway

    Lipid metabolism; fatty acid beta-oxidation.
  • Involvement in disease

    Defects in HADHB 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 three enzyme activities of the TFP complex.
  • Sequence similarities

    Belongs to the thiolase family.
  • Cellular localization

    Mitochondrion.
  • Information by UniProt

Images

  • ab158632 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab158632 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab158632.
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