2-Enoyl-Coenzyme A Recombinant Protein | HADHB recombinant protein
Recombinant Human 2-Enoyl-Coenzyme A (CoA) Hydratase, Beta
Gene Names
HADHB; ECHB; MTPB; MSTP029; TP-BETA
Purity
Greater than 90.0% as determined by SDS-PAGE.
Synonyms
2-Enoyl-Coenzyme A; Recombinant Human 2-Enoyl-Coenzyme A (CoA) Hydratase; Beta; 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; HADHB recombinant protein
Host
E Coli
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.
Sterile Filtered clear solution.
Sequence
MGSSHHHHHH SSGLVPRGSH MGSAAPAVQT 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.
Sequence Length
474
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.
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
NCBI and Uniprot Product Information
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
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
NCBI Official Synonym Symbols
ECHB; MTPB; MSTP029; TP-BETA
NCBI Protein Information
trifunctional enzyme subunit beta, mitochondrial
UniProt Protein Name
Trifunctional enzyme subunit beta, mitochondrial
Protein Family
UniProt Gene Name
HADHB
UniProt Entry Name
ECHB_HUMAN
Uniprot Description
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
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Product Notes
The HADHB hadhb (Catalog #AAA140855) is a Recombinant Protein produced from E Coli and is intended for research purposes only. The product is available for immediate purchase. The amino acid sequence is listed below: 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.. It is sometimes possible for the material contained within the vial of "2-Enoyl-Coenzyme A, Recombinant Protein" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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