Human HADHB Recombinant Protein (RPPB1791)
- SKU:
- RPPB1791
- Product Type:
- Recombinant Protein
- Species:
- Human
- Uniprot:
- P55084
- Research Area:
- Enzymes
Description
| Product Name: | Human HADHB Recombinant Protein |
| Product Code: | RPPB1791 |
| Size: | 20µg |
| Species: | Human |
| Target: | HADHB |
| Synonyms: | 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. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered clear solution. |
| Formulation: | HADHB protein solution (0.5mg/ml) containing 20mM phosphate (pH8.0) and 10% glycerol. |
| Stability: | Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles. |
| Purity: | Greater than 90.0% as determined by SDS-PAGE. |
| Amino Acid 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 |
2-Enoyl-CoenzymeA (CoA) Hydratase, Beta (HADHB) is the beta subunit of the mitochondrialtrifunctional protein, that catalyzes the last 3 phases of mitochondrialbeta-oxidation of long chain fatty acids. HADHB binds RNA and reduces thestability of various mRNAs. Mutations in HADHB cause trifunctional proteindeficiency.
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.
| UniProt Protein Function: | 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. |
| UniProt Protein Details: | Protein type:Lipid Metabolism - fatty acid; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Transferase; Lipid Metabolism - fatty acid elongation in mitochondria; EC 2.3.1.16 Chromosomal Location of Human Ortholog: 2p23 Cellular Component: mitochondrial envelope; mitochondrial outer membrane; mitochondrion; endoplasmic reticulum; mitochondrial inner membrane; fatty acid beta-oxidation multienzyme complex Molecular Function:protein binding; acyl-CoA binding; enoyl-CoA hydratase activity; long-chain-3-hydroxyacyl-CoA dehydrogenase activity; protein complex binding; long-chain-enoyl-CoA hydratase activity; NAD binding; acetyl-CoA C-acyltransferase activity; 3-hydroxyacyl-CoA dehydrogenase activity Biological Process: fatty acid beta-oxidation; phospholipid metabolic process; glycerophospholipid biosynthetic process; cellular lipid metabolic process Disease: Trifunctional Protein Deficiency |
| NCBI Summary: | This gene encodes 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. The encoded 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. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2013] |
| UniProt Code: | P55084 |
| NCBI GenInfo Identifier: | 116241345 |
| NCBI Gene ID: | 3032 |
| NCBI Accession: | P55084.3 |
| UniProt Secondary Accession: | P55084,O14969, Q53TA6, Q96C77, Q9H3F5, Q9T2V8, B2RB16 B4E2W0, |
| UniProt Related Accession: | P55084 |
| Molecular Weight: | 474 |
| NCBI Full Name: | Trifunctional enzyme subunit beta, mitochondrial |
| NCBI 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; beta-ketothiolase; acetyl-CoA acyltransferase; 2-enoyl-Coenzyme A (CoA) hydratase, beta subunit; 3-ketoacyl-Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein, beta subunit; hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit |
| 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�� |
| UniProt Entry Name: | ECHB_HUMAN |
