ACADVL, Polyclonal Antibody

ACAD6; LCACD; VLCAD

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

Polyclonal

Rabbit

The antibody detects endogenous levels of total ACADVL protein.

Antigen affinity purification.

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

0.9 mg/ml (lot specific)

Store at -20 degree C

Small volumes of anti-ACADVL 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.

The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms.

Total protein Ab

Western Blot (WB), Immunohistochemistry (IHC)

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

Gel: 8%SDS-PAGE Lysate: 40ug Hela cell Primary antibody: 1/200 dilution Secondary antibody dilution: 1/8000 Exposure time: 5 seconds

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

72,927 Da

acyl-CoA dehydrogenase, very long chain

very long-chain specific acyl-CoA dehydrogenase, mitochondrial

Very long-chain specific acyl-CoA dehydrogenase, mitochondrial

VLCAD; VLCAD  [Similar Products]

ACADV_HUMAN

The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008]

ACADVL: Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons. Defects in ACADVL are the cause of acyl-CoA dehydrogenase very long chain deficiency (ACADVLD). ACADVLD is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form, with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and rare cardiomyopathy; and an ***** form, with isolated skeletal muscle involvement, rhabdomyolysis, and myoglobinuria, usually triggered by exercise or fasting. Belongs to the acyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 1.3.8.9; Oxidoreductase; Mitochondrial; Lipid Metabolism - fatty acid

Chromosomal Location of Human Ortholog: 17p13.1

Cellular Component: mitochondrion; mitochondrial matrix; cytoplasm; mitochondrial inner membrane; nucleolus; nucleus

Molecular Function: acyl-CoA dehydrogenase activity; FAD binding; long-chain-acyl-CoA dehydrogenase activity

Biological Process: cellular protein metabolic process; unfolded protein response, activation of signaling protein activity; fatty acid beta-oxidation; epithelial cell differentiation; unfolded protein response; thermoregulation; cellular lipid metabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; negative regulation of fatty acid biosynthetic process; negative regulation of fatty acid oxidation; energy derivation by oxidation of organic compounds

Disease: Acyl-coa Dehydrogenase, Very Long-chain, Deficiency Of

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.