Description

  • Product name

    Recombinant Human BOLA3 protein (His tag)
  • Purity

    > 95 % SDS-PAGE.
    ab201887 was purified using conventional chromatography.
  • Expression system

    Escherichia coli
  • Accession

  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      MGSSHHHHHHSSGLVPRGSHMGSMAAWSPAAAAPLLRGIRGLPLHHRMFA TQTEGELRVTQILKEKFPRATAIKVTDISGGCGAMYEIKIESEEFKEKRT VQQHQMVNQALKEEIKEMHGLRIFTSVPKR
    • Predicted molecular weight

      15 kDa including tags
    • Amino acids

      1 to 107
    • Tags

      His tag N-Terminus
    • Additional sequence information

      NCBI Accession No.: NP_997717

Associated products

Specifications

Our Abpromise guarantee covers the use of ab201887 in the following tested applications.

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

  • Applications

    SDS-PAGE

    Mass Spectrometry

  • Mass spectrometry

    MALDI-TOF
  • Form

    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 7.4
    Constituents: 89% PBS, 10% Glycerol

General Info

  • Alternative names

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

Images

  • 15% SDS-PAGE analysis of ab201887 (3 µg)

References

ab201887 has not yet been referenced specifically in any publications.

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