Recombinant
RabMAb

Anti-HMGCL antibody [EPR15550] - N-terminal (ab187648)

Overview

  • Product name
    Anti-HMGCL antibody [EPR15550] - N-terminal
    See all HMGCL primary antibodies
  • Description
    Rabbit monoclonal [EPR15550] to HMGCL - N-terminal
  • Tested applications
    Suitable for: IHC-P, Flow Cyt, WBmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Synthetic peptide (the amino acid sequence is considered to be commercially sensitive) within Human HMGCL aa 1-100 (N terminal). The exact sequence is proprietary.
    Database link: P35914

  • Positive control
    • A431 cell line lysate, Human fetal kidney and fetal liver lysates; Human liver tissue; 293 cells.
  • General notes

    This product is a recombinant rabbit monoclonal antibody.

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents

Properties

Applications

Our Abpromise guarantee covers the use of ab187648 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
IHC-P 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
Flow Cyt 1/20. ab172730-Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody.
WB 1/1000 - 1/10000. Detects a band of approximately 34 kDa (predicted molecular weight: 34 kDa).

Target

  • Function
    Involved in the catabolism of branched amino acids such as leucine.
  • Tissue specificity
    Fibroblasts, liver and lymphoblasts.
  • Pathway
    Metabolic intermediate metabolism; (S)-3-hydroxy-3-methylglutaryl-CoA degradation; acetoacetate from (S)-3-hydroxy-3-methylglutaryl-CoA: step 1/1.
  • Involvement in disease
    Defects in HMGCL are the cause of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD) [MIM:246450]; also known as hydroxymethylglutaricaciduria or HL deficiency. An autosomal recessive disease affecting ketogenesis and L-leucine catabolism. The disease usually appears in the first year of life after a fasting period and its clinical acute symptoms include vomiting, seizures, metabolic acidosis, hypoketotic hypoglycemia and lethargy. These symptoms sometimes progress to coma, with fatal outcome in some cases.
  • Sequence similarities
    Belongs to the HMG-CoA lyase family.
  • Cellular localization
    Mitochondrion matrix.
  • Information by UniProt
  • Database links
  • Alternative names
    • 3 hydroxy 3 methylglutaryl CoA lyase antibody
    • 3 hydroxy 3 methylglutaryl Coenzyme A lyase antibody
    • 3 hydroxymethyl 3 methylglutaryl Coenzyme A lyase (hydroxymethylglutaricaciduria) antibody
    • 3 hydroxymethyl 3 methylglutaryl Coenzyme A lyase antibody
    • 3-hydroxy-3-methylglutarate-CoA lyase antibody
    • HL antibody
    • HMG CoA lyase antibody
    • HMG CoA Lyase Deficiency antibody
    • HMG-CoA lyase antibody
    • HMGCL antibody
    • HMGCL_HUMAN antibody
    • Hydroxymethylglutaricaciduria antibody
    • Hydroxymethylglutaryl CoA lyase antibody
    • Hydroxymethylglutaryl CoA lyase mitochondrial antibody
    • Hydroxymethylglutaryl-CoA lyase antibody
    • Mitochondrial 3 hydroxy 3 methylglutaryl CoA lyase antibody
    • mitochondrial antibody
    • MS725 antibody
    • OTTHUMP00000044830 antibody
    see all

Images

  • Flow cytometric analysis of 2% paraformaldehyde fixed 293 cells labeling HMGCL with ab187648 at 1/20 (red) or Rabbit monoclonal IgG (green) followed by Goat anti rabbit IgG (FITC) antibody at 1/150.

  • Immunohistochemistry analysis of paraffin embedded Human liver tissue labeling HMGCL with ab187648 at 1/500 followed by a ready to use HRP polymer for Rabbit IgG and counterstained with hematoxylin.

  • Anti-HMGCL antibody [EPR15550] - N-terminal (ab187648) at 1/5000 dilution + Human fetal liver tissue lysate at 10 µg

    Secondary
    Anti-Rabbit IgG (HRP), specific to the non-reduced form of IgG at 1/1000 dilution

    Predicted band size : 34 kDa
  • Anti-HMGCL antibody [EPR15550] - N-terminal (ab187648) at 1/1000 dilution + Human fetal kidney tissue at 10 µg

    Secondary
    Anti-Rabbit IgG (HRP), specific to the non-reduced form of IgG at 1/1000 dilution

    Predicted band size : 34 kDa
  • Anti-HMGCL antibody [EPR15550] - N-terminal (ab187648) at 1/1000 dilution + A431 cell line lysate at 20 µg

    Secondary
    Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated antibody at 1/1000 dilution

    Predicted band size : 34 kDa

References

ab187648 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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