Recombinant human ACADSB protein
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.
Product Specifications
Product Name Alternative
Expression System
E.coli
Antigen Species
Human
Tag
His-Tag
Applications
SDS-PAGE
Concentration
0.5 mg/mL (determined by Bradford assay)
Purity
> 90% by SDS-PAGE
Molecular Weight
46.4 kDa (424aa)
Additionnal Information
References & Citations
Arden K C., et al. (1995) Genomics. 25: 743-745.; ; Korman S H., et al. (2005) Clin Chem. 51: 610-617.
Storage Conditions
Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.
Formulation
Liquid in. 20 mM Tris-HCl buffer (pH 8.0) containing 0.1M NaCl, 10% glycerol,1mM DTT
Scientific Category
Signal Transduction
NCBI Accession Number
NP_001600
Uniprot Accession Number
P45954
Species
Human
AA Sequence
Frequently Asked Questions
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