Human ACADS Recombinant Protein (RPPB1319)
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주문- SKU:
- RPPB1319
- Product type:
- Recombinant Protein
- Size:
- 10ug
- Species:
- Human
- Target:
- ACADS
- Synonyms:
- ACAD3
- Synonyms:
- SCAD
- Synonyms:
- EC 13992
- Synonyms:
- Short-chain specific acyl-CoA dehydrogenase
- Source:
- Escherichia Coli
- Uniprot:
- P16219
Description
| Product Name: | Human ACADS Recombinant Protein |
| Product Code: | RPPB1319 |
| Size: | 10µg |
| Species: | Human |
| Target: | ACADS |
| Synonyms: | ACAD3, SCAD, EC 1.3.99.2, Short-chain specific acyl-CoA dehydrogenase, mitochondrial, Butyryl-CoA dehydrogenase, ACADS. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile filtered colorless solution. |
| Formulation: | The protein contains 20mM Tris buffer pH-8, 1mM DTT, 20% glycerol and 100mM NaCl. |
| 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 95.0% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MGSSHHHHHH SSGLVPRGSH MLHTIYQSVE LPETHQMLLQ TCRDFAEKEL FPIAAQVDKE HLFPAAQVKK MGGLGLLAMD VPEELGGAGL DYLAYAIAME EISRGCASTG VIMSVNNSLY LGPILKFGSK EQKQAWVTPF TSGDKIGCFA LSEPGNGSDA GAASTTARAE GDSWVLNGTK AWITNAWEAS AAVVFASTDR ALQNKSISAF LVPMPTPGLT LGKKEDKLGI RGSSTANLIF EDCRIPKDSI LGEPGMGFKI AMQTLDMGRI GIASQALGIA QTALDCAVNY AENRMAFGAP LTKLQVIQFK LADMALALES ARLLTWRAAM LKDNKKPFIK EAAMAKLAAS EAATAISHQA IQILGGMGYV TEMPAERHYR DARITEIYEG TSEIQRLVIA GHLLRSYRS |
ACADS is a tetrameric mitochondrial flavoprotein, which is part of the acyl-CoA dehydrogenase family. ACADS catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. Mutations in ACADS have been associated with Short Chain Acyl-CoA Dehydrogenase Deficiency.
ACADS Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 409 amino acids (25-412 a.a.) and having a molecular mass of 44 kDa. The ACADS protein is fused to a 20 amino acid His Tag at N-terminus and purified by standard chromatogrpahy techniques.
| UniProt Protein Function: | ACADS: Defects in ACADS are the cause of acyl-CoA dehydrogenase short-chain deficiency (ACADSD). It is an autosomal recessive disorder resulting in acute acidosis and muscle weakness in infants, and a form of lipid-storage myopathy in adults. Belongs to the acyl-CoA dehydrogenase family. |
| UniProt Protein Details: | Protein type:EC 1.3.8.1; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Oxidoreductase; Carbohydrate Metabolism - butanoate; Lipid Metabolism - fatty acid Chromosomal Location of Human Ortholog: 12q24.31 Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial membrane; nucleus Molecular Function:acyl-CoA dehydrogenase activity; butyryl-CoA dehydrogenase activity; FAD binding; acyl-CoA binding Biological Process: response to starvation; fatty acid beta-oxidation; response to glucocorticoid stimulus; cellular lipid metabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; butyrate catabolic process; protein homotetramerization Disease: Acyl-coa Dehydrogenase, Short-chain, Deficiency Of |
| NCBI Summary: | This gene encodes a tetrameric mitochondrial flavoprotein, which is a member of the acyl-CoA dehydrogenase family. This enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Mutations in this gene have been associated with short-chain acyl-CoA dehydrogenase (SCAD) deficiency. Alternative splicing results in two variants which encode different isoforms. [provided by RefSeq, Oct 2014] |
| UniProt Code: | P16219 |
| NCBI GenInfo Identifier: | 113019 |
| NCBI Gene ID: | 35 |
| NCBI Accession: | P16219.1 |
| UniProt Secondary Accession: | P16219,P78331, |
| UniProt Related Accession: | P16219 |
| Molecular Weight: | 412 |
| NCBI Full Name: | Short-chain specific acyl-CoA dehydrogenase, mitochondrial |
| NCBI Synonym Full Names: | acyl-CoA dehydrogenase, C-2 to C-3 short chain |
| NCBI Official Symbol: | ACADS |
| NCBI Official Synonym Symbols: | SCAD; ACAD3 |
| NCBI Protein Information: | short-chain specific acyl-CoA dehydrogenase, mitochondrial; butyryl-CoA dehydrogenase; unsaturated acyl-CoA reductase; acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain; mitochondrial short-chain specific acyl-CoA dehydrogenase |
| UniProt Protein Name: | Short-chain specific acyl-CoA dehydrogenase, mitochondrial |
| UniProt Synonym Protein Names: | Butyryl-CoA dehydrogenase |
| Protein Family: | Short-chain specific acyl-CoA dehydrogenase |
| UniProt Gene Name: | ACADS |
| UniProt Entry Name: | ACADS_HUMAN |
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