Recombinant human HADHB protein

HADHB is the beta subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. This protein can also bind RNA and decreases the stability of some mRNAs. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. Mutations in this gene result in trifunctional protein deficiency. Recombinant human HADHB protein, fused to His-tag at N-terminus, was expressed in E. coli.

Product Specifications

Product Name Alternative

Trifunctional enzyme subunit beta mitochondrial, Trifunctional enzyme subunit beta, mitochondrial, ECHB, MSTP029, MTPB, TP-BETA

Expression System

E.coli

Antigen Species

Human

Tag

His-Tag

Applications

SDS-PAGE, Denatured

Concentration

0.5 mg/mL (determined by Bradford assay)

Purity

> 90% by SDS-PAGE

Molecular Weight

49.9 kDa (464aa)

Additionnal Information

HADHB, Trifunctional enzyme subunit beta mitochondrial, Trifunctional enzyme subunit beta, mitochondrial, ECHB, MSTP029, MTPB, TP-BETA, ATGP2941-10 µg, ATGP2941-20 µg, ATGP2941-50 µg, ATGP2941-100 µg, ATGP2941-250 µg, ATGP2941-500 µg, ATGP2941-1 mg, ATGP2941-10, ATGP2941-20, ATGP2941-50, ATGP2941-100, ATGP2941-250, ATGP2941-500, ATGP2941-1

References & Citations

Purevsuren J., et al. (2009) Mol Genet Metab, PMID 19699128

Storage Conditions

Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.

Formulation

Liquid in. 20 mM Tris-HCl buffer (pH 8.0) containing 10% glycerol

Scientific Category

Metabolism

NCBI Accession Number

NP_000174

Uniprot Accession Number

P55084

Species

Human

AA Sequence

AAPAVQT KTKKTLAKPN IRNVVVVDGV RTPFLLSGTS YKDLMPHDLA RAALTGLLHR TSVPKEVVDY IIFGTVIQEV KTSNVAREAA LGAGFSDKTP AHTVTMACIS ANQAMTTGVG LIASGQCDVI VAGGVELMSD VPIRHSRKMR KLMLDLNKAK SMGQRLSLIS KFRFNFLAPE LPAVSEFSTS ETMGHSADRL AAAFAVSRLE QDEYALRSHS LAKKAQDEGL LSDVVPFKVP GKDTVTKDNG IRPSSLEQMA KLKPAFIKPY GTVTAANSSF LTDGASAMLI MAEEKALAMG YKPKAYLRDF MYVSQDPKDQ LLLGPTYATP KVLEKAGLTM NDIDAFEFHE AFSGQILANF KAMDSDWFAE NYMGRKTKVG LPPLEKFNNW GGSLSLGHPF GATGCRLVMA AANRLRKEGG QYGLVAACAA GGQGHAMIVE AYPK