Overview

  • Product name
  • Description
    Rabbit polyclonal to LMBRD1
  • Tested applications
    Suitable for: WB, ELISA, IHC-Pmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse, Rat
  • Immunogen

    Synthetic peptide conjugated to KLH, from the C terminus of Human LMBRD1.

  • Positive control
    • Human breast or myenteric plexus (colon) tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab117641 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 Use a concentration of 1 - 2 µg/ml. Predicted molecular weight: 61 kDa.
ELISA Use at an assay dependent concentration.
IHC-P Use a concentration of 5 µg/ml.

Target

  • Function
    Probable lysosomal cobalamin transporter. Required to export cobalamin from lysosomes allowing its conversion to cofactors. Isoform 3 may play a role in the assembly of hepatitis delta virus (HDV).
  • Tissue specificity
    Isoform 3 is expressed in liver.
  • Involvement in disease
    Defects in LMBRD1 are the cause of methylmalonic aciduria and homocystinuria type cblF (MMAFHC) [MIM:277380]; also known as homocystinuria-megaloblastic anemia complementation type F. MMAFHC is a disorder of cobalamin metabolism characterized by decreased levels of the coenzymes adenosylcobalamin (AdoCbl) and methylcobalamin (MeCbl). It is due to accumulation of free cobalamin in lysosomes, thus hindering its conversion to cofactors. Clinical features include developmental delay, stomatitis, glossitis, seizures and methylmalonic aciduria responsive to vitamin B12.
  • Sequence similarities
    Belongs to the LIMR family. LMBRD1 subfamily.
  • Post-translational
    modifications
    N-glycosylated.
  • Cellular localization
    Lysosome membrane.
  • Information by UniProt
  • Database links
  • Alternative names
    • HDAg-L-interacting protein NESI antibody
    • LMBD1_HUMAN antibody
    • LMBR1 domain-containing protein 1 antibody
    • lmbrd1 antibody
    • Nuclear export signal-interacting protein antibody
    • Probable lysosomal cobalamin transporter antibody
    see all

Images

  • Human breast, Formalin-Fixed, Paraffin-Embedded tissue, stained with ab117641 at 5µg/ml.
  • Human Myenteric Plexus (colon), Formalin-Fixed, Paraffin-Embedded tissue, stained with ab117641 at 5µg/ml.

References

ab117641 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

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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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