Human ACADL Recombinant Protein (RPPB5767)
- SKU:
- RPPB5767
- Product Type:
- Recombinant Protein
- Species:
- Human
- Uniprot:
- P28330
- Research Area:
- Enzymes
Description
| Product Name: | Human ACADL Recombinant Protein |
| Product Code: | RPPB5767 |
| Size: | 10µg |
| Species: | Human |
| Target: | ACADL |
| Synonyms: | Acyl-CoA dehydrogenase long chain, Acyl-Coenzyme A dehydrogenase long chain, LCAD, ong-chain specific acyl-CoA dehydrogenase mitochondrial, ACAD4, EC 1.3.99.13. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered colorless solution. |
| Formulation: | The ACADL solution (1mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 0.15M NaCl, 1mM DTT 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 85% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MGSSHHHHHH SSGLVPRGSH MGGEERLETP SAKKLTDIGI RRIFSPEHDI FRKSVRKFFQ EEVIPHHSEW EKAGEVSREV WEKAGKQGLL GVNIAEHLGG IGGDLYSAAI VWEEQAYSNC SGPGFSIHSG IVMSYITNHG SEEQIKHFIP QMTAGKCIGA IAMTEPGAGS DLQGIKTNAK KDGSDWILNG SKVFISNGSL SDVVIVVAVT NHEAPSPAHG ISLFLVENGM KGFIKGRKLH KMGLKAQDTA ELFFEDIRLP ASALLGEENK GFYYIMKELP QERLLIADVA ISASEFMFEE TRNYVKQRKA FGKTVAHLQT VQHKLAELKT HICVTRAFVD NCLQLHEAKR LDSATACMAK YWASELQNSV AYDCVQLHGG WGYMWEYPIA KAYVDARVQP IYGGTNEIMK ELIAREIVFD K |
ACADL is a homotetramer belonging to the acyl-CoA dehydrogenase family. ACADL takes part in the catabolism of fatty acids and amino acids and is a key source of energy for the heart and skeletal muscle. Mutation in the ACADL gene results in non-ketotic hypoglycemia and hypotonia (muscle weakness).
ACADL Human Recombinant produced in E.coli is a single, non-glycosylated polypeptide chain containing 421 amino acids (31-430) and having a molecular mass of 46.7 kDa.ACADL is fused to a 21 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.
| UniProt Protein Function: | ACADL: Defects in ACADL are a cause of acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD). An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy; a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy; an adult form, with isolated skeletal muscle involvement, rhabdomyolysis and myoglobinuria, usually triggered by exercise or fasting. Belongs to the acyl-CoA dehydrogenase family. |
| UniProt Protein Details: | Protein type:Mitochondrial; Lipid Metabolism - fatty acid; EC 1.3.8.8; Oxidoreductase Chromosomal Location of Human Ortholog: 2q34 Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial membrane; cytoplasm Molecular Function:palmitoyl-CoA oxidase activity; acyl-CoA dehydrogenase activity; FAD binding; acyl-CoA binding; long-chain-acyl-CoA dehydrogenase activity Biological Process: carnitine metabolic process, CoA-linked; fatty acid beta-oxidation; cellular lipid catabolic process; carnitine catabolic process; thermoregulation; cellular lipid metabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; negative regulation of fatty acid oxidation; negative regulation of fatty acid biosynthetic process; long-chain fatty acid catabolic process; protein homotetramerization |
| NCBI Summary: | The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. [provided by RefSeq, Jul 2008] |
| UniProt Code: | P28330 |
| NCBI GenInfo Identifier: | 223590148 |
| NCBI Gene ID: | 33 |
| NCBI Accession: | P28330.2 |
| UniProt Related Accession: | P28330 |
| Molecular Weight: | |
| NCBI Full Name: | Long-chain specific acyl-CoA dehydrogenase, mitochondrial |
| NCBI Synonym Full Names: | acyl-CoA dehydrogenase long chain |
| NCBI Official Symbol: | ACADL�� |
| NCBI Official Synonym Symbols: | LCAD; ACAD4�� |
| NCBI Protein Information: | long-chain specific acyl-CoA dehydrogenase, mitochondrial |
| UniProt Protein Name: | Long-chain specific acyl-CoA dehydrogenase, mitochondrial |
| Protein Family: | Long-chain specific acyl-CoA dehydrogenase |
| UniProt Gene Name: | ACADL�� |
| UniProt Entry Name: | ACADL_HUMAN |
