ACADVL, Polyclonal Antibody

VLCAD; ACADVL; Very long-chain specific acyl-CoA dehydrogenase; mitochondrial

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

Polyclonal

IgG

Rabbit

Affinity Purified

Colorless liquid
30 ug (0.5 mg/ml) affinity purified rabbit anti-ACAD-9 polyclonal antibody in phosphate buffered saline (PBS); pH 7.2; containing 30% glycerol; 0.5% BSA; 5 mM EDTA; 5 mM EDTA and 0.03% proclin

Store at -20 degree C for 12 months.
The antibody solution should be gently mixed before use.

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.

Polyclonal Antibody to detect ACADVL in human; mouse and rat samples.
Acyl-CoA dehydrogenase; very long chain (ACADVL) is a member of the acyl-CoA dehydrogenases family. ACADVL is an inner mitochondrial membrane protein that catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. A deficiency of ACADVL reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy.

ACADVL

Western Blot (WB)

WB: ~1:1000
FACS: ~1:10-1:50
IHC: ~1:50-1:100
IF: ~1:10-1:50

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]

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; Lipid Metabolism - fatty acid; Mitochondrial; Oxidoreductase

Chromosomal Location of Human Ortholog: 17p13.1

Cellular Component: cytosol; mitochondrial matrix; mitochondrion; nucleolus; nucleus

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

Biological Process: energy derivation by oxidation of organic compounds; epithelial cell differentiation; fatty acid beta-oxidation; fatty acid beta-oxidation using acyl-CoA dehydrogenase; negative regulation of fatty acid biosynthetic process; negative regulation of fatty acid oxidation; thermoregulation

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

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