Overview

  • Product name

  • Description

    Rabbit polyclonal to BOLA3
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-Pmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat
  • Immunogen

    Recombinant fragment corresponding to BOLA3 aa 18-105.
    Sequence:

    GLPLHHRMFATQTEGELRVTQILKEKFPRATAIKVTDISGGCGAMYEIKI ESEEFKEKRTVQQHQMVNQALKEEIKEMHGLRIFTSVP


    Database link: Q53S33

  • Positive control

    • Human kidney tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab185339 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 1/200 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

Target

  • Tissue specificity

    Widely expressed.
  • Involvement in disease

    Defects in BOLA3 are the cause of multiple mitochondrial dysfunctions syndrome type 2 (MMDS2) [MIM:614299]. A severe disorder of systemic energy metabolism, resulting in weakness, respiratory failure, lack of neurologic development, lactic acidosis, hyperglycinemia and early death. Some patients show failure to thrive, pulmonary hypertension, hypotonia and irritability. Biochemical features include severe combined deficiency of the 2-oxoacid dehydrogenases, defective lipoic acid synthesis and reduction in activity of mitochondrial respiratory chain complexes.
  • Sequence similarities

    Belongs to the bolA/yrbA family.
  • Cellular localization

    Secreted. Could be secreted via a non-classical export pathway.
  • Information by UniProt
  • Database links

  • Alternative names

    • BolA-like protein 3 antibody
    • BOLA3 antibody
    • BOLA3_HUMAN antibody
    • MMDS2 antibody

Images

  • Immunohistochemistry analysis of Human kidney tissue labeling BOLA3 with ab185339 at 1/200 dilution. 

References

ab185339 has not yet been referenced specifically in any publications.

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