ACADSB, Peptide

For Research Use Only. Not for use in diagnostic procedures.

50 ug (0.5 mg/ml) in phosphate buffered saline (PBS), pH 7.2, containing 50% glycerol, 1% BSA and 0.02% thimerosal.
Appearance: Colorless liquid

0.5 mg/ml (lot specific)

At -20 degree C
Shelf Life: 12 months

Small volumes of ACADSB peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Antibodies & Supporting Tools; Blocking Peptides

Western Blot (WB)

The peptide is used for blocking the antibody activity of ACADSB. It usually blocks the antibody activity completely in Western blot analysis by incubating the peptide with equal volume of antibody for 30-60 minutes at 37 degree C.

47,874 Da

acyl-Coenzyme A dehydrogenase, short/branched chain

short/branched chain specific acyl-CoA dehydrogenase, mitochondrial

Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial

SBCAD; 2-MEBCAD; 2-methylbutyryl-CoA dehydrogenase  [Similar Products]

ACDSB_MOUSE

ACADSB: Has greatest activity toward short branched chain acyl- CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl- CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent. Defects in ACADSB are the cause of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD); also known as 2-methylbutyryl-CoA dehydrogenase deficiency or 2- methylbutyryl glycinuria. SBCADD is an autosomal recessive disorder and consists of a defect in catabolism of L-isoleucine which is characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine. Affected individuals have seizures and psychomotor delay as the main clinical features. Belongs to the acyl-CoA dehydrogenase family.

Protein type: Oxidoreductase; Lipid Metabolism - fatty acid; Mitochondrial; EC 1.3.8.5; Amino Acid Metabolism - valine, leucine and isoleucine degradation

Cellular Component: mitochondrion

Molecular Function: short-branched-chain-acyl-CoA dehydrogenase activity; acyl-CoA dehydrogenase activity; FAD binding; electron carrier activity; oxidoreductase activity, acting on the CH-CH group of donors; oxidoreductase activity

Biological Process: acyl-CoA metabolic process; metabolic process; lipid metabolic process; fatty acid metabolic process

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