ACADM, Polyclonal Antibody

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

Polyclonal

Rabbit

Human

Affinity purified

PBS, pH 7.4 with 0.02% Sodium Azide

1.0 mg/ml (lot specific)

This product is stable for several weeks at 4 degree C as an undiluted liquid. Dilute only prior to immediate use. For extended storage, aliquot contents and freeze at -20 degree C or below. Avoid cycles of freezing and thawing. Expiration date is one (1) year from date of receipt.

Small volumes of anti-ACADM 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.

Total protein Ab

Western Blot (WB), Immunohistochemistry (IHC)

47,020 Da

acyl-CoA dehydrogenase, C-4 to C-12 straight chain

medium-chain specific acyl-CoA dehydrogenase, mitochondrial

Medium-chain specific acyl-CoA dehydrogenase, mitochondrial

MCAD  [Similar Products]

ACADM_HUMAN

This gene encodes the medium-chain specific (C4 to C12 straight chain) acyl-Coenzyme A dehydrogenase. The homotetramer enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Defects in this gene cause medium-chain acyl-CoA dehydrogenase deficiency, a disease characterized by hepatic dysfunction, fasting hypoglycemia, and encephalopathy, which can result in infantile death. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

ACADM: This enzyme is specific for acyl chain lengths of 4 to 16. Defects in ACADM are the cause of acyl-CoA dehydrogenase medium-chain deficiency (ACADMD). It is an autosomal recessive disease which causes fasting hypoglycemia, hepatic dysfunction, and encephalopathy, often resulting in death in infancy. Belongs to the acyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Lipid Metabolism - fatty acid; Carbohydrate Metabolism - propanoate; Oxidoreductase; EC 1.3.8.7; Other Amino Acids Metabolism - beta-alanine

Chromosomal Location of Human Ortholog: 1p31

Cellular Component: mitochondrion; axon; mitochondrial matrix; nucleus

Molecular Function: identical protein binding; acyl-CoA dehydrogenase activity; FAD binding

Biological Process: carnitine metabolic process, CoA-linked; fatty acid beta-oxidation; medium-chain fatty acid catabolic process; cellular lipid metabolic process; medium-chain fatty acid metabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; carnitine biosynthetic process

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

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