Description

  • Product name

    Recombinant Human NFU1 protein
  • Purity

    > 90 % SDS-PAGE.

  • Expression system

    Escherichia coli
  • Accession

  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      MGSSHHHHHHSSGLVPRGSHMGSGAAAVAAGLRRRFCHMLKNPYTIKKQP LHQFVQRPLFPLPAAFYHPVRYMFIQTQDTPNPNSLKFIPGKPVLETRTM DFPTPAAAFRSPLARQLFRIEGVKSVFFGPDFITVTKENEELDWNLLKPD IYATIMDFFASGLPLVTEETPSGEAGSEEDDEVVAMIKELLDTRIRPTVQ EDGGDVIYKGFEDGIVQLKLQGSCTSCPSSIITLKNGIQNMLQFYIPEVE GVEQVMDDESDEKEANSP
    • Predicted molecular weight

      30 kDa including tags
    • Amino acids

      10 to 254
    • Tags

      His tag N-Terminus
    • Additional sequence information

      Full length mature protein

Specifications

Our Abpromise guarantee covers the use of ab187452 in the following tested applications.

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

  • Applications

    Mass Spectrometry

    SDS-PAGE

  • 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: 7.40
    Constituents: 89% PBS, 10% Glycerol, 0.02% DTT

General Info

  • Alternative names

    • 0610006G17Rik
    • CGI 33
    • HIRA interacting protein 5
    • HIRA-interacting protein 5
    • Hirip5
    • Histone cell cycle regulation defective interacting protein 5
    • Iron sulfur cluster scaffold protein
    • MGC142252
    • MGC142254
    • mHIRIP5
    • mitochondrial
    • Nfu
    • NFU1
    • NFU1 iron sulfur cluster scaffold homolog
    • NFU1 iron sulfur cluster scaffold homolog (S. cerevisiae)
    • NFU1 iron sulfur cluster scaffold homolog, mitochondrial
    • NFU1 iron-sulfur cluster scaffold homolog
    • NFU1_HUMAN
    • NifU
    • NIFUC
    • OTTHUMP00000214438
    • OTTHUMP00000214439
    • OTTHUMP00000214465
    • OTTHUMP00000214466
    • OTTMUSP00000038171
    • OTTMUSP00000038172
    see all
  • Function

    Iron-sulfur cluster scaffold protein which can assemble [4Fe-2S] clusters and deliver them to target proteins.
  • Tissue specificity

    Ubiquitous. Expression in adult lung is weak compared to fetal lung.
  • Involvement in disease

    Defects in NFU1 are the cause of multiple mitochondrial dysfunctions syndrome type 1 (MMDS1) [MIM:605711]. 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 NifU family.
  • Developmental stage

    Expressed in embryo and adult.
  • Cellular localization

    Mitochondrion. Cytoplasm > cytosol.
  • Information by UniProt

Images

  • 15% SDS-PAGE analysis of 3µg ab187452.

References

ab187452 has not yet been referenced specifically in any publications.

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