Recombinant human ACADSB protein
€0.00
In stock
SKU
ATGP1628
Description
Short/branched chain specific acyl-CoA dehydrogenase (ACADSB), is a member of the acyl-CoA dehydrogenase family of enzymes that 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 and has the highest affinity for (s) -2-methylbutyryl-CoA, isobutyryl-CoA and 2-methylhexanoyl-CoA as substrates. Defects in ACADSB are the cause of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD), an autosomal recessive disorder characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine. Recombinant human ACADSB protein, fused to His-tag at N-terminus, was expressed in E. coli and purified by using conventional chromatography techniques.Alternative Names
Short/branched chain specific acyl-CoA dehydrogenase mitochondrial, Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial, 2-MEBCAD, ACAD7, SBCADConcentration
0.5mg/ml (determined by Bradford assay)Concentration
Liquid in. 20mM Tris-HCl buffer (pH 8.0) containing 0.1M NaCl, 10% glycerol,1mM DTTStorage: Can be stored at +2C to +8C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.
Short/branched chain specific acyl-CoA dehydrogenase (ACADSB), is a member of the acyl-CoA dehydrogenase family of enzymes that 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 and has the highest affinity for (s) -2-methylbutyryl-CoA, isobutyryl-CoA and 2-methylhexanoyl-CoA as substrates. Defects in ACADSB are the cause of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD), an autosomal recessive disorder characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine. Recombinant human ACADSB protein, fused to His-tag at N-terminus, was expressed in E. coli and purified by using conventional chromatography techniques.Alternative Names
Short/branched chain specific acyl-CoA dehydrogenase mitochondrial, Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial, 2-MEBCAD, ACAD7, SBCADConcentration
0.5mg/ml (determined by Bradford assay)Concentration
Liquid in. 20mM Tris-HCl buffer (pH 8.0) containing 0.1M NaCl, 10% glycerol,1mM DTTStorage: Can be stored at +2C to +8C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.
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