Rabbit Polyclonal ACADVL Antibody

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     Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane: Hela cells, Primary antibody: P04251(ACADVL Antibody) at dilution 1/200, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 5 seconds    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using P04251(ACADVL Antibody) at dilution 1/15. (Original magnification: ×200)    

  
  

The ACADVL antibody is a crucial tool for studying very long-chain acyl-CoA dehydrogenase (VLCAD), an enzyme encoded by the ACADVL gene. VLCAD is essential for mitochondrial fatty acid β-oxidation, specifically catalyzing the initial step in breaking down very long-chain fatty acids (VLCFAs) into acetyl-CoA for energy production. Mutations in ACADVL lead to VLCAD deficiency, a rare autosomal recessive disorder characterized by metabolic crises, cardiomyopathy, hepatic dysfunction, and muscle weakness.

ACADVL antibodies are primarily used in research and diagnostics to detect and quantify VLCAD protein levels. They enable techniques like Western blotting, immunohistochemistry, and immunofluorescence to assess tissue-specific expression, subcellular localization, and potential deficiencies in patient samples. These antibodies aid in understanding the molecular mechanisms underlying VLCAD deficiency and evaluating therapeutic interventions, such as gene therapy or enzyme replacement strategies.

Commercially available ACADVL antibodies are typically raised against specific epitopes of the human VLCAD protein, with validation in various species and sample types. Researchers rely on them to study fatty acid metabolism disorders, model disease phenotypes, and validate CRISPR-edited or knockout cell lines. Their specificity and sensitivity are critical for distinguishing VLCAD from other acyl-CoA dehydrogenases, ensuring accurate diagnosis and mechanistic insights into metabolic pathways.