Anti-ACADVL antibody (ab54698)

Overview

• Product nameAnti-ACADVL antibodySee all ACADVL primary antibodies ...
• Description
  Mouse monoclonal to ACADVL
• Tested applicationsFlow Cyt, WB, IHC-P more details
• Species reactivity
  Reacts with: Human
  
• Immunogen

  Recombinant fragment: AAALAGTMRG IIAKAVDHAT NRTQFGEKIH NFGLIQEKLA RMVMLQYVTE SMAYMVSANM DQGATDFQIE AAISKIFGSE AAWKVTDECI , corresponding to amino acids 345-435 of ACADVL Recombinant fragment: AAALAGTMRG IIAKAVDHAT NRTQFGEKIH NFGLIQEKLA RMVMLQYVTE SMAYMVSANM DQGATDFQIE AAISKIFGSE AAWKVTDECI , corresponding to amino acids 345-435 of Human ACADVL

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Properties

• FormLiquid
• Storage instructionsStore at +4°C short term (1-2 weeks). Aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
• Storage bufferPreservative: None
  Constituents: PBS, pH 7.2
• Concentration information loading...
• PurityProtein G purified
• Clonality Monoclonal
• IsotypeIgG2b
• Light chain typekappa
• Research Areas
  • Metabolism
  • Types of disease
  • Cancer

  • Metabolism
  • Pathways and Processes
  • Redox metabolism
  • Fatty acid oxidation

  • Metabolism
  • Pathways and Processes
  • Metabolic signaling pathways
  • Lipid and lipoprotein metabolism
  • Fatty acids

  • Metabolism
  • Pathways and Processes
  • Metabolic signaling pathways
  • Lipid and lipoprotein metabolism
  • Lipid metabolism

  • Metabolism
  • Pathways and Processes
  • Mitochondrial Metabolism
  • Mitochondrial markers

  • Cancer
  • Cancer Metabolism
  • Metabolic signaling pathway
  • Metabolism of lipids and lipoproteins

  • Signal Transduction
  • Metabolism
  • Mitochondrial

  • Cardiovascular
  • Lipids / Lipoproteins
  • Fatty Acids
  • Metabolism

Applications

Target

• FunctionActive toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accomodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons.
• PathwayLipid metabolism; mitochondrial fatty acid beta-oxidation.
• Involvement in diseaseDefects in ACADVL are the cause of acyl-CoA dehydrogenase very long chain deficiency (ACADVLD) [MIM:201475]. 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.
• Sequence similaritiesBelongs to the acyl-CoA dehydrogenase family.
• Cellular localizationMitochondrion inner membrane.

Target information above from: UniProt accession P49748 The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010) .

Information by UniProt
• Database links
  • Entrez Gene: 37 Human
  • Omim: 609575 Human
  • SwissProt: P49748 Human
  • Unigene: 437178 Human

• Alternative names
  ACAD 6 antibodyACAD6 antibodyACADV_HUMAN antibody

  Acadvl antibodyAcyl CoA dehydrogenase very long chain antibodyAcyl Coenzyme A dehydrogenase very long chain antibodyLCACD antibodymitochondrial antibodyVery long chain specific acyl CoA dehydrogenase antibodyVery long chain specific acyl CoA dehydrogenase mitochondrial antibodyVery long-chain specific acyl-CoA dehydrogenase antibodyVLCAD antibody

  see all

Anti-ACADVL antibody images

• 

  Western blot - ACADVL antibody (ab54698)
  
  
  Predicted band size : 70 kDa
  Observed band size : 70 kDa
  
• 

  Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - ACADVL antibody (ab54698)
  ab54698 used in immunohistochemistry at 3ug/ml in formalin fixed and paraffin embedded human heart.
• 

  Immunocytochemistry/ Immunofluorescence-ACADVL antibody(ab54698)
  ICC/IF image of ab54698 stained HeLa cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab54698, 5µg/ml) overnight at +4°C. The secondary antibody (green) was ab96879, DyLight® 488 goat anti-mouse IgG (H+L) used at a 1/250 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.
  
  
• 

  Flow Cytometry - Anti-ACADVL antibody (ab54698)
  Overlay histogram showing HeLa cells stained with ab54698 (red line). The cells were fixed with 80% methanol (5 min) and then permeabilized with 0.1% PBS-Tween for 20 min. The cells were then incubated in 1x PBS / 10% normal goat serum / 0.3M glycine to block non-specific protein-protein interactions followed by the antibody (ab54698, 1µg/1x10⁶ cells) for 30 min at 22ºC. The secondary antibody used was DyLight® 488 goat anti-mouse IgG (H+L) (ab96879) at 1/500 dilution for 30 min at 22ºC. Isotype control antibody (black line) was mouse IgG2b [PLPV219] (ab91366, 2µg/1x10⁶ cells) used under the same conditions. Acquisition of >5,000 events was performed. This antibody gave a positive signal in HeLa cells fixed with 4% paraformaldehyde (10 min)/permeabilized with 0.1% PBS-Tween for 20 min used under the same conditions.