SCAD; ACAD3

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

Polyclonal

Rabbit

The antibody detects endogenous levels of total ACADS protein.

Antigen affinity purification.

Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol.

0.8 mg/ml (lot specific)

Store at -20 degree C

Small volumes of anti-ACADS antibody 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.

This gene encodes a a tetrameric mitochondrial flavoprotein, which is a member of the acyl-CoA dehydrogenase family. This enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Mutations in this gene have been associated with Short Chain Acyl-CoA Dehydrogenase Deficiency. It is an autosomal recessive disorder resulting in acute acidosis and muscle weakness in infants, and a form of lipid-storage myopathy in *****s.

Total protein Ab

Western Blot (WB), Immunohistochemistry (IHC)

Western blotting: 1:200-1:1000
Immunohistochemistry: 1:25-1:100

Gel: 10%SDS-PAGE Lysates (from left to right): Rat kidney tissue Amount of lysate: 40ug per lane Primary antibody: 1/350 dilution Secondary antibody dilution: 1/8000 Exposure time: 90 seconds

Immunohistochemical analysis of paraffin-embedded Human cervical cancer tissue using at dilution 1/15.

44,297 Da

acyl-CoA dehydrogenase, C-2 to C-3 short chain

short-chain specific acyl-CoA dehydrogenase, mitochondrial

Short-chain specific acyl-CoA dehydrogenase, mitochondrial

SCAD??[Similar Products]

ACADS_HUMAN

This gene encodes a tetrameric mitochondrial flavoprotein, which is a member of the acyl-CoA dehydrogenase family. This enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Mutations in this gene have been associated with short-chain acyl-CoA dehydrogenase (SCAD) deficiency. Alternative splicing results in two variants which encode different isoforms. [provided by RefSeq, Oct 2014]

ACADS: Defects in ACADS are the cause of acyl-CoA dehydrogenase short-chain deficiency (ACADSD). It is an autosomal recessive disorder resulting in acute acidosis and muscle weakness in infants, and a form of lipid-storage myopathy in *****s. Belongs to the acyl-CoA dehydrogenase family.

Protein type: Mitochondrial; EC 1.3.8.1; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Oxidoreductase; Carbohydrate Metabolism - butanoate; Lipid Metabolism - fatty acid

Chromosomal Location of Human Ortholog: 12q24.31

Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial membrane; nucleus

Molecular Function: acyl-CoA dehydrogenase activity; butyryl-CoA dehydrogenase activity; FAD binding; acyl-CoA binding

Biological Process: response to starvation; fatty acid beta-oxidation; response to glucocorticoid stimulus; cellular lipid metabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; butyrate catabolic process; protein homotetramerization

Disease: Acyl-coa Dehydrogenase, Short-chain, Deficiency Of

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