Human ACADM Recombinant Protein (RPPB5768)
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주문- SKU:
- RPPB5768
- Product type:
- Recombinant Protein
- Size:
- 10ug
- Species:
- Human
- Target:
- ACADM
- Synonyms:
- ACADM
- Synonyms:
- ACAD1
- Synonyms:
- CAD
- Synonyms:
- MCADH
- Source:
- Escherichia Coli
- Uniprot:
- P11310
Description
| Product Name: | Human ACADM Recombinant Protein |
| Product Code: | RPPB5768 |
| Size: | 10µg |
| Species: | Human |
| Target: | ACADM |
| Synonyms: | ACADM, ACAD1, CAD, MCADH, MCAD, EC=1.3.99.3, Medium-chain specific acyl-CoA dehydrogenase, mitochondrial, FLJ18227, FLJ93013, FLJ99884. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile filtered colorless solution. |
| Formulation: | The ACADM (0.5mg/ml) protein solution containing 20mM Tris-HCl pH-7.5, 0.1M NaCl & 20% 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 MKANRQREPG LGFSFEFTEQ QKEFQATARK FAREEIIPVA AEYDKTGEYP VPLIRRAWEL GLMNTHIPEN CGGLGLGTFD ACLISEELAY GCTGVQTAIE GNSLGQMPII IAGNDQQKKK YLGRMTEEPL MCAYCVTEPG AGSDVAGIKT KAEKKGDEYI INGQKMWITN GGKANWYFLL ARSDPDPKAP ANKAFTGFIV EADTPGIQIG RKELNMGQRC SDTRGIVFED VKVPKENVLI GDGAGFKVAM GAFDKTRPVV AAGAVGLAQR ALDEATKYAL ERKTFGKLLV EHQAISFMLA EMAMKVELAR MSYQRAAWEV DSGRRNTYYA SIAKAFAGDI ANQLATDAVQ ILGGNGFNTE YPVEKLMRDA KIYQIYEGTS QIQRLIVARE HIDKYKN |
ACADM enzyme is essential for the degradation a certain group of fats called medium-chain fatty acids. ACADM is essential for converting specific fatty acids to energy, mainly during fasting periods. ACADM functions in mitochondria, the energy-producing centers within cells. ACADM is localized in the mitochondria of numerous tissue types, predominantly the liver.
ACADM Recombinant Human produced in E.Coli is a single, non-glycosylated polypeptide chain containing 417 amino acids (26-421 a.a.) and having a molecular mass of 45.9 kDa. The ACADM is fused to a 21 amino acid His-Tag at N-terminus and purified by proprietary chromatographic techniques.
| UniProt Protein Function: | ACADM: This enzyme is specific for acyl chain lengths of 4 to 16. Defects in ACADM are the cause of acyl-CoA dehydrogenase medium-chain deficiency (ACADMD). It is an autosomal recessive disease which causes fasting hypoglycemia, hepatic dysfunction, and encephalopathy, often resulting in death in infancy. Belongs to the acyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:EC 1.3.8.7; Mitochondrial; Carbohydrate Metabolism - propanoate; Oxidoreductase; Lipid Metabolism - fatty acid; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Other Amino Acids Metabolism - beta-alanine Chromosomal Location of Human Ortholog: 1p31 Cellular Component: mitochondrion; axon; mitochondrial matrix; nucleus Molecular Function:acyl-CoA dehydrogenase activity; identical protein binding; FAD binding Biological Process: carnitine metabolic process, CoA-linked; fatty acid beta-oxidation; medium-chain fatty acid catabolic process; cellular lipid metabolic process; medium-chain fatty acid metabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; carnitine biosynthetic process Disease: Acyl-coa Dehydrogenase, Medium-chain, Deficiency Of |
| NCBI Summary: | This gene encodes the medium-chain specific (C4 to C12 straight chain) acyl-Coenzyme A dehydrogenase. The homotetramer enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Defects in this gene cause medium-chain acyl-CoA dehydrogenase deficiency, a disease characterized by hepatic dysfunction, fasting hypoglycemia, and encephalopathy, which can result in infantile death. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008] |
| UniProt Code: | P11310 |
| NCBI GenInfo Identifier: | 113017 |
| NCBI Gene ID: | 34 |
| NCBI Accession: | P11310.1 |
| UniProt Related Accession: | P11310 |
| Molecular Weight: | |
| NCBI Full Name: | Medium-chain specific acyl-CoA dehydrogenase, mitochondrial |
| NCBI Synonym Full Names: | acyl-CoA dehydrogenase medium chain |
| NCBI Official Symbol: | ACADM |
| NCBI Official Synonym Symbols: | MCAD; ACAD1; MCADH |
| NCBI Protein Information: | medium-chain specific acyl-CoA dehydrogenase, mitochondrial |
| UniProt Protein Name: | Medium-chain specific acyl-CoA dehydrogenase, mitochondrial |
| UniProt Gene Name: | ACADM |
| UniProt Entry Name: | ACADM_HUMAN |
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