ACADM, Antibody

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

Rabbit

1.0mg/ml (lot specific)

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.

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]

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

Chromosomal Location of Human Ortholog: 1p31.1

Cellular Component: axon; mitochondrial matrix; mitochondrion; nucleus; peroxisome

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

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

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

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