Mouse anti-Human ACADVL Monoclonal Antibody | anti-ACADVL antibody

ACADVL (Acyl Coenzyme A Dehydrogenase Very Long Chain, Acyl-CoA Dehydrogenase Very Long Chain, ACAD6, LCACD, Very Long-chain Specific Acyl-CoA Dehydrogenase Mitochondrial, VLCAD) APC

Cat. Num. AAA6135132

• Gene Names: ACADVL; ACAD6; LCACD; VLCAD
• Reactivity: Human
• Applications: ELISA, Immunohistochemistry, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: ACADVL; Monoclonal Antibody; ACADVL (Acyl Coenzyme A Dehydrogenase Very Long Chain; Acyl-CoA Dehydrogenase Very Long Chain; ACAD6; LCACD; Very Long-chain Specific Acyl-CoA Dehydrogenase Mitochondrial; VLCAD) APC; anti-ACADVL antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG2b,k
• Clone Number: 5D3
• Specificity: Recognizes human ACADVL.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Allophycocyanin (APC).

• Applicable Applications for anti-ACADVL antibody: ELISA (EIA), Immunohistochemistry (IHC) Paraffin, Western Blot (WB)
• Application Notes: IHC-P: 3ug/ml; Applications are based on unconjugated antibody.
• Immunogen: Partial recombinant corresponding to aa345-434 from human ACADVL (NP_000009) with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: AAALAGTMRGIIAKAVDHATNRTQFGEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISKIFGSEAAWKVTDECI
• Conjugate: APC
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: APC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western Blot detection against Immunogen (35.64kD).)

Western Blot (WB)

(ACADVL monoclonal antibody Western Blot analysis of ACADVL expression in Hela NE.)

Immunohistochemistry (IHC)

(Immunoperoxidase of monoclonal antibody to ACADVL on formalin-fixed paraffin-embedded human heart. [antibody concentration 3ug/ml])

Testing Data

(Detection limit for recombinant GST tagged ACADVL is ~0.03ng/ml as a capture antibody.)

Product Categories/Family for anti-ACADVL antibody

Antibodies; Enzymes; Dehydrogenase

References

1. Mild mitochondrial uncoupling does not affect mitochondrial biogenesis but downregulates pyruvate carboxylase in adipocytes: role for triglyceride content reduction. De Pauw A, Demine S, Tejerina S, Dieu M, Delaive E, Kel A, Renard P, Raes M, Arnould T.Am J Physiol Endocrinol Metab. 2012 May; 302(9):E1123-41. Epub 2012 Feb 21.

NCBI and Uniprot Product Information

• NCBI GI #: 4557235
• NCBI GeneID: 37
• NCBI Accession #: NP_000009
• NCBI GenBank Nucleotide #: NM_000018
• UniProt Accession #: P49748; O76056; Q8WUL0; B4DEB6; F5H2A9
• Molecular Weight: 68.5 kDa (636aa)
• NCBI Official Full Name: very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 1
• NCBI Official Synonym Full Names: acyl-CoA dehydrogenase very long chain
• NCBI Official Symbol: ACADVL
• NCBI Official Synonym Symbols: ACAD6; LCACD; VLCAD
• NCBI Protein Information: very long-chain specific acyl-CoA dehydrogenase, mitochondrial
• UniProt Protein Name: Very long-chain specific acyl-CoA dehydrogenase, mitochondrial
• Protein Family: Very long-chain specific acyl-CoA dehydrogenase
• UniProt Gene Name: ACADVL
• UniProt Synonym Gene Names: VLCAD; VLCAD
• UniProt Entry Name: ACADV_HUMAN

NCBI Description

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]

Uniprot Description

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 adult 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: Oxidoreductase; EC 1.3.8.9; Lipid Metabolism - fatty acid; Mitochondrial

Chromosomal Location of Human Ortholog: 17p13.1

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

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

Biological Process: unfolded protein response, activation of signaling protein activity; cellular protein metabolic process; fatty acid beta-oxidation; epithelial cell differentiation; unfolded protein response; cellular lipid metabolic process; thermoregulation; 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