Recombinant
RabMAb

Anti-NSDHL antibody [EPR14490] (ab190353)

Overview

  • Product name
    Anti-NSDHL antibody [EPR14490]
    See all NSDHL primary antibodies
  • Description
    Rabbit monoclonal [EPR14490] to NSDHL
  • Tested applications
    Suitable for: WB, IHC-P, ICC/IFmore details
  • Species reactivity
    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide (the amino acid sequence is considered to be commercially sensitive) within Human NSDHL aa 150-250. The exact sequence is proprietary.
    Database link: Q15738

  • Positive control
    • A431, HeLa and Human fetal brain lysates. Human liver and Human adenocarcinoma of endometrium tissue. U87-MG 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 ab190353 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
WB 1/1000 - 1/10000. Detects a band of approximately 38 kDa (predicted molecular weight: 42 kDa).
IHC-P 1/50 - 1/100. Perform heat mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol.
ICC/IF 1/50.

Target

  • Tissue specificity
    Brain, heart, liver, lung, kidney, skin and placenta.
  • Pathway
    Steroid biosynthesis; zymosterol biosynthesis; zymosterol from lanosterol: step 4/6.
  • Involvement in disease
    Defects in NSDHL are the cause of congenital hemidysplasia with ichthyosiform erythroderma and limb defects (CHILD) [MIM:308050]. CHILD is an X-linked dominant disorder of lipid metabolism with disturbed cholesterol biosynthesis, which typically results in male lethality. Clinically, it is characterized by congenital, unilateral, ichthyosisform erythroderma with striking lateralization, sharp midline demarcation, and ipsilateral limb defects and hypoplasia of the body. Limbs defects range from hypoplasia of digits or ribs to complete amelia, often including scoliosis.
    Defects in NSDHL are the cause of CK syndrome (CKS) [MIM:300831]. CKS is a disorder characterized by mild to severe cognitive impairment, seizures, microcephaly, cerebral cortical malformations, dysmorphic facial features, and thin body habitus.
  • Sequence similarities
    Belongs to the 3-beta-HSD family.
  • Cellular localization
    Membrane.
  • Information by UniProt
  • Database links
  • Alternative names
    • decarboxylating antibody
    • H105E3 antibody
    • H105e3 protein antibody
    • NAD(P) dependent steroid dehydrogenase like antibody
    • NSDHL antibody
    • NSDHL_HUMAN antibody
    • Protein H105e3 antibody
    • SDR31E1 antibody
    • Short chain dehydrogenase/reductase family 31E member 1 antibody
    • Sterol 4 alpha carboxylate 3 dehydrogenase decarboxylating antibody
    • Sterol-4-alpha-carboxylate 3-dehydrogenase antibody
    • XAP104 antibody
    see all

Images

  • All lanes : Anti-NSDHL antibody [EPR14490] (ab190353) at 1/10000 dilution

    Lane 1 : A431 cell lysate
    Lane 2 : HeLa cell lysate
    Lane 3 : Human fetal brain lysate

    Lysates/proteins at 20 µg per lane.

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

    Predicted band size : 42 kDa
    Observed band size : 38 kDa (why is the actual band size different from the predicted?)
  • Immunohistochemical analysis of paraffin-embedded Human adenocarcinoma of endometrium tissue labeling NSDHL with ab190353 at 1/100 dilution. Secondary antibody HRP Polymer for Rabbit IgG prediluted. Counter stain Hematoxylin. Negative control also shown.

  • Immunohistochemical analysis of paraffin-embedded Human liver tissue labeling NSDHL with ab190353 at 1/100 dilution. Secondary antibody HRP Polymer for Rabbit IgG prediluted. Counter stain Hematoxylin. Negative control also shown.

  • Immunofluorescence analysis of U87-MG cells, labeling NSDHL (red) with ab190353 at 1/50 dilution. Alexa Fluor®555-conjugated goat anti-rabbit IgG was used as a secondary antibody at 1/200 dilution. Nuclei were counterstained with DAPI (blue).

References

ab190353 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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