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ACADVL Antibody (N-term) Blocking Peptide

Synthetic peptide

     
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Product Information
Primary Accession P49748
Clone Names 80924028
Additional Information
Gene ID 37
Other Names Very long-chain specific acyl-CoA dehydrogenase, mitochondrial, VLCAD, ACADVL, VLCAD
Target/Specificity The synthetic peptide sequence used to generate the antibody AP6597a was selected from the N-term region of human ACADVL. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.
Format Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed.
StorageMaintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.
PrecautionsThis product is for research use only. Not for use in diagnostic or therapeutic procedures.
Protein Information
Name ACADVL (HGNC:92)
Function Very long-chain specific acyl-CoA dehydrogenase is one of the acyl-CoA dehydrogenases that catalyze the first step of mitochondrial fatty acid beta-oxidation, an aerobic process breaking down fatty acids into acetyl-CoA and allowing the production of energy from fats (PubMed:7668252, PubMed:9461620, PubMed:18227065, PubMed:9839948, PubMed:9599005). The first step of fatty acid beta-oxidation consists in the removal of one hydrogen from C-2 and C-3 of the straight-chain fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl- CoA (PubMed:7668252, PubMed:9461620, PubMed:18227065, PubMed:9839948). Among the different mitochondrial acyl-CoA dehydrogenases, very long- chain specific acyl-CoA dehydrogenase acts specifically on acyl-CoAs with saturated 12 to 24 carbons long primary chains (PubMed:21237683, PubMed:9839948).
Cellular Location Mitochondrion inner membrane; Peripheral membrane protein
Tissue Location Predominantly expressed in heart and skeletal muscle (at protein level). Also detected in kidney and liver (at protein level).
Research Areas
Citations (0)
citation

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Background

ACADVL 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 its gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy.

References

Gobin-Limballe,S., Am. J. Hum. Genet. 81 (6), 1133-1143 (2007)Zia,A., J. Inherit. Metab. Dis. 30 (5), 817 (2007)

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$ 277.78
Cat# BP6597a
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