ACADM, Polyclonal Antibody

Goat Anti-ACADM, Biotinylated Antibody; ACADM; acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain; RP4-682C21.1; ACAD1; MCAD; MCADH; medium-chain acyl-CoA dehydrogenase; FLJ18227; FLJ93013; FLJ99884; medium-chain specific acyl-CoA dehydrogenase; ACADM antibody; acyl-Coenzyme A dehydrogenase; C-4 to C-12 straight chain antibody; RP4-682C21.1 antibody; ACAD1 antibody; MCAD antibody; MCADH antibody; medium-chain acyl-CoA dehydrogenase antibody; FLJ18227 antibody; FLJ93013 antibody; FLJ99884 antibody; medium-chain specific acyl-CoA dehydrogenase antibody

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

Polyclonal

Goat

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

100ug specific antibody in 200ul (lot specific)

Aliquot and store at -20 degree C. Minimize freezing and thawing.

Manufactured in an ISO 9001:2008 Certified Laboratory.

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.

Peptide ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

Peptide ELISA: Antibody detection limit dilution 1: 32000.
Immunohistochemistry: In paraffin embedded Human Liver shows mitochondrial staining in hepatocytes. Recommended concentration, 3-5ug/ml.
Western Blot: Approx 45kDa band observed in Human Kidney and Heart lysates (calculated MW of 46.6kDa according to NP_000007.1). Recommended for use at 0. 01-0.1ug/ml.

Staining (0. 05ug/ml) of Human Heart lysate (RIPA buffer, 35ug total protein per lane). Primary incubated for 1 hour. Detected by western blot using chemiluminescence.

(3.8ug/ml) staining of paraffin embedded Human Liver. Steamed antigen retrieval with citrate buffer pH 6, AP-staining.

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

Chromosomal Location of Human Ortholog: 1p31

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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