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| Catalog # | Availability | Size | Quantity | Unit Price | Save For Later Wish List | |
|---|---|---|---|---|---|---|
| ENZ0014-2 | 2 µg | $120.00 |
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| ENZ0014-10 | 10 µg | $290.00 | ||||
| ENZ0014-1 | 1 mg | $8,320.00 |
Product Overview | |
| Name | ACADS Human |
|---|---|
| Description | |
| Acyl-Coenzyme A Dehydrogenase C-2 to C-3 Human Recombinant | |
| Accession (Primary) | P16219 |
| Synonyms | |
| Short/branched chain specific acyl-CoA dehydrogenase mitochondrial, SBCAD, 2-methyl branched chain acyl-CoA dehydrogenase, 2-MEBCAD, 2-methylbutyryl-coenzyme A dehydrogenase, 2-methylbutyryl-CoA dehydrogenase, ACADSB, ACAD7, SBCAD, 2-MEBCAD. | |
| Introduction | |
| Short/branched chain specific acyl-CoA dehydrogenase (ACADSB) belongs to the acyl-CoA dehydrogenase family of enzymes which catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. ACADSB catalyzes the degradation of L-isoleucine while having the highest affinity for (s)-2-methylbutyryl-CoA, isobutyryl-CoA and 2-methylhexanoyl-CoA as substrates. ACADSB may use valproyl-CoA as substrate. ACADSB gene defects cause the short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD), which is an autosomal recessive disorder characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine. | |
| Source | |
| Escherichia Coli. | |
| Physical Appearance | |
| Sterile Filtered clear solution. | |
| Formulation | |
| The ACADSB solution (0.5mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 0.1M NaCl, 10% glycerol and 1mM DTT. | |
| 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 MGSHM KSSQS EALLNITNNG IHFAPLQTFT DEEMMIKSSV KKFAQEQIAP LVSTMDENSK MEKSVIQGLF QQGLMGIEVD PEYGGTGASF LSTVLVIEEL AKVDASVAVF CEIQNTLINT LIRKHGTEEQ KATYLPQLTT EKVGSFCLSE AGAGSDSFAL KTRADKEGDY YVLNGSKMWI SSAEHAGLFL VMANVDPTIG YKGITSFLVD RDTPGLHIGK PENKLGLRAS STCPLTFENV KVPEANILGQ IGHGYKYAIG SLNEGRIGIA AQMLGLAQGC FDYTIPYIKE RIQFGKRLFD FQGLQHQVAH VATQLEAARL LTYNAARLLE AGKPFIKEAS MAKYYASEIA GQTTSKCIEW MGGVGYTKDY PVEKYFRDAK IGTIYEGASN IQLNTIAKHI DAEY. | |
| Precautions | |
| ACADS Human is for research use only and not for use in diagnostic or therapeutic procedures. | |
Target Information: ( P16219 ) | |
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