Date published: 2026-5-15

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AUH Antibody (B-8): sc-518216

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Datasheets
  • AUH Antibody (B-8) is a mouse monoclonal IgG1 κ provided at 200 µg/ml
  • specific for an epitope mapping between amino acids 321-339 of AUH of human origin
  • recommended for detection of AUH of mouse, rat and human origin by WB, IP, IF and ELISA
  • Anti-AUH Antibody (B-8) is available conjugated to agarose for IP; HRP for WB, IHC(P) and ELISA; and to either phycoerythrin or FITC for IF, IHC(P) and FCM
  • also available conjugated to Alexa Fluor® 488, Alexa Fluor® 546, Alexa Fluor® 594 or Alexa Fluor® 647 for WB (RGB), IF, IHC(P) and FCM, and for use with RGB fluorescent imaging systems, such as iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
  • also available conjugated to Alexa Fluor® 680 or Alexa Fluor® 790 for WB (NIR), IF and FCM; for use with Near-Infrared (NIR) detection systems, such as LI-COR®Odyssey®, iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
  • m-IgG Fc BP-HRP, m-IgG1 BP-HRP and m-IgGκ BP-HRP are the preferred secondary detection reagents for AUH Antibody (B-8) for WB applications. These reagents are now offered in bundles with AUH Antibody (B-8) (see ordering information below).
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    AUH Antibody (B-8) is a mouse monoclonal IgG1 kappa light chain antibody that detects AUH in mouse, rat, and human samples through applications such as western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). AUH antibody (B-8) is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. AUH, also known as AU-binding protein or mitochondrial methylglutaconyl-CoA hydratase, consists of 339 amino acids and belongs to the enoyl-CoA hydratase/isomerase family. AUH plays a crucial role in the amino acid degradation pathway by catalyzing the conversion of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl-CoA and water, a process vital for energy metabolism and proper breakdown of certain amino acids. AUH is localized in mitochondria, where AUH has shown very low enoyl-CoA hydratase activity, and exists as two isoforms due to alternative splicing, forming a homohexamer structure. Deficiencies in AUH can lead to 3-methylglutaconic aciduria type 1 (MGA1), a metabolic disorder characterized by severe symptoms, including coma, psychomotor retardation, and metabolic acidosis, highlighting AUH′s importance in maintaining metabolic health.

    For Research Use Only. Not Intended for Diagnostic or Therapeutic Use.

    Alexa Fluor® is a trademark of Molecular Probes Inc., OR., USA

    LI-COR® and Odyssey® are registered trademarks of LI-COR Biosciences

    AUH Antibody (B-8) References:

    1. Characterisation and mitochondrial localisation of AUH, an AU-specific RNA-binding enoyl-CoA hydratase.  |  Brennan, LE., et al. 1999. Gene. 228: 85-91. PMID: 10072761
    2. Crystal structure of human AUH protein, a single-stranded RNA binding homolog of enoyl-CoA hydratase.  |  Kurimoto, K., et al. 2001. Structure. 9: 1253-63. PMID: 11738050
    3. 3-Methylglutaconic aciduria type I is caused by mutations in AUH.  |  IJlst, L., et al. 2002. Am J Hum Genet. 71: 1463-6. PMID: 12434311
    4. Mutations in the AUH gene cause 3-methylglutaconic aciduria type I.  |  Ly, TB., et al. 2003. Hum Mutat. 21: 401-7. PMID: 12655555
    5. 3-methylglutaconic aciduria type I in a boy with fever-associated seizures.  |  Illsinger, S., et al. 2004. Pediatr Neurol. 30: 213-5. PMID: 15033206
    6. Biochemical characterization of human 3-methylglutaconyl-CoA hydratase and its role in leucine metabolism.  |  Mack, M., et al. 2006. FEBS J. 273: 2012-22. PMID: 16640564
    7. AU-rich RNA-binding induces changes in the quaternary structure of AUH.  |  Kurimoto, K., et al. 2009. Proteins. 75: 360-72. PMID: 18831052
    8. AUH, a gene encoding an AU-specific RNA binding protein with intrinsic enoyl-CoA hydratase activity.  |  Nakagawa, J., et al. 1995. Proc Natl Acad Sci U S A. 92: 2051-5. PMID: 7892223
    9. A 20-amino-acid autonomous RNA-binding domain contained in an enoyl-CoA hydratase.  |  Nakagawa, J. and Moroni, C. 1997. Eur J Biochem. 244: 890-9. PMID: 9108262

    Ordering Information

    Product NameCatalog #UNITPriceQtyFAVORITES

    AUH Antibody (B-8)

    sc-518216
    200 µg/ml
    $322.00

    AUH Antibody (B-8): m-IgG Fc BP-HRP Bundle

    sc-551270
    200 µg Ab; 10 µg BP
    $361.00

    AUH Antibody (B-8): m-IgG1 BP-HRP Bundle

    sc-551268
    200 µg Ab; 20 µg BP
    $361.00

    AUH Antibody (B-8): m-IgGκ BP-HRP Bundle

    sc-551269
    200 µg Ab; 40 µg BP
    $361.00

    AUH Antibody (B-8) AC

    sc-518216 AC
    500 µg/ml, 25% agarose
    $424.00

    AUH Antibody (B-8) HRP

    sc-518216 HRP
    200 µg/ml
    $322.00

    AUH Antibody (B-8) FITC

    sc-518216 FITC
    200 µg/ml
    $336.00

    AUH Antibody (B-8) PE

    sc-518216 PE
    200 µg/ml
    $349.00

    AUH Antibody (B-8) Alexa Fluor® 488

    sc-518216 AF488
    200 µg/ml
    $364.00

    AUH Antibody (B-8) Alexa Fluor® 546

    sc-518216 AF546
    200 µg/ml
    $364.00

    AUH Antibody (B-8) Alexa Fluor® 594

    sc-518216 AF594
    200 µg/ml
    $364.00

    AUH Antibody (B-8) Alexa Fluor® 647

    sc-518216 AF647
    200 µg/ml
    $364.00

    AUH Antibody (B-8) Alexa Fluor® 680

    sc-518216 AF680
    200 µg/ml
    $364.00

    AUH Antibody (B-8) Alexa Fluor® 790

    sc-518216 AF790
    200 µg/ml
    $364.00