Product Name
2-Enoyl-Coenzyme A (HADHB), Recombinant Protein
Full Product Name
Recombinant Human 2-Enoyl-Coenzyme A (CoA) Hydratase, Beta
Product Synonym Names
HADHB Human; 2-Enoyl-Coenzyme A (CoA) Hydratase, Beta Human Recombinant; Hydroxyacyl-CoA Dehydrogenase/3-Ketoacyl-CoA Thiolase/Enoyl-CoA Hydratase (Trifunctional Protein) Beta Subunit; Hydroxyacyl-Coenzyme A Dehydrogenase/3-Ketoacyl-Coenzyme A Thiolase/Enoyl-Coenzyme A Hydratase (Trifunctional Protein) Beta Subunit; TP-BETA; 3-Ketoacyl-Coenzyme A (CoA) Thiolase Of Mitochondrial Trifunctional Protein Beta Subunit; 2-Enoyl-Coenzyme A (CoA) Hydratase Beta Subunit; Trifunctional Enzyme Subunit Beta Mitochondrial; Mitochondrial Trifunctional Protein; Acetyl-CoA Acyltransferase; Beta-Ketothiolase; Beta Subunit; EC 2.3.1.16; EC 2.3.1; MSTP029; ECHB; MTPB
Product Gene Name
HADHB recombinant protein
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence
MGSSHHH HHH SSGLVPRGSH MGSAAP AVQT 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.
3D Structure
ModBase 3D Structure for P55084
Purity/Purification
Greater than 90.0% as determined by SDS-PAGE.
Form/Format
HADHB protein solution (0.5mg/ml) containing 20mM phosphate (pH8.0) and 10% glycerol.
Sterile Filtered clear solution.
Other Notes
Small volumes of HADHB recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
HADHB recombinant protein
Introduction: 2-Enoyl-Coenzyme A (CoA) Hydratase, Beta (HADHB) is the beta subunit of the mitochondrial trifunctional protein, that catalyzes the last 3 phases of mitochondrial beta-oxidation of long chain fatty acids. HADHB binds RNA and reduces the stability of various mRNAs. Mutations in HADHB cause trifunctional protein deficiency.
Description: HADHB Human Recombinant produced in E Coli is a single, non-glycosylated polypeptide chain containing 464 amino acids (34-474 a.a) and having a molecular mass of 49.9kDa. HADHB is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.
Product Categories/Family for HADHB recombinant protein
ENZYMES
NCBI/Uniprot data below describe general gene information for HADHB. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000174.1
[Other Products]
NCBI GenBank Nucleotide #
NM_000183.2
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UniProt Primary Accession #
P55084
[Other Products]
UniProt Secondary Accession #
O14969; Q53TA6; Q96C77; Q9H3F5; Q9T2V8; B2RB16; B4E2W0[Other Products]
UniProt Related Accession #
P55084[Other Products]
Molecular Weight
48,880 Da
NCBI Official Full Name
trifunctional enzyme subunit beta, mitochondrial isoform 1
NCBI Official Synonym Full Names
hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit
NCBI Official Symbol
HADHB??[Similar Products]
NCBI Official Synonym Symbols
ECHB; MTPB; MSTP029; TP-BETA
??[Similar Products]
NCBI Protein Information
trifunctional enzyme subunit beta, mitochondrial
UniProt Protein Name
Trifunctional enzyme subunit beta, mitochondrial
UniProt Synonym Protein Names
TP-betaIncluding the following 1 domains:3-ketoacyl-CoA thiolase (EC:2.3.1.16)Alternative name(s):Acetyl-CoA acyltransferase; Beta-ketothiolase
Protein Family
Trifunctional enzyme
UniProt Gene Name
HADHB??[Similar Products]
UniProt Entry Name
ECHB_HUMAN
UniProt Comments for HADHB
HADHB: Defects in HADHB are a cause of trifunctional protein deficiency (TFP deficiency). 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. Belongs to the thiolase family.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 2.3.1.16; Lipid Metabolism - fatty acid; Lipid Metabolism - fatty acid elongation in mitochondria; Transferase
Chromosomal Location of Human Ortholog: 2p23
Cellular Component: endoplasmic reticulum; mitochondrial envelope; mitochondrial inner membrane; mitochondrial outer membrane
Molecular Function: 3-hydroxyacyl-CoA dehydrogenase activity; enoyl-CoA hydratase activity; protein binding
Biological Process: fatty acid beta-oxidation
Disease: Trifunctional Protein Deficiency
Precautions
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Disclaimer
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