| Field | Specification |
|---|---|
| Mfr No | |
| Enzyme Type | |
| Expression System | |
| Form | Liquid |
| Formulation | |
| Product Type | |
| Purity | |
| Source | Escherichia Coli. |
| Species | |
| Storage | |
| Target |
Product Description
Acyl-CoA Dehydrogenase, Short Chain Human Recombinant
Overview
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.
Specifications
| Purity | Greater than 90.0% as determined by SDS-PAGE. |
|---|---|
| Formulation | The ACADSB solution (0.5mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 0.1M NaCl, 10% glycerol and 1mM DTT. |
| Storage | 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. |
| Expression System | Escherichia Coli. |