Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      MAATARRGWGAAAVAAGLRRRFCHMLKNPYTIKKQPLHQFVQRPLFPLPA AFYHPVRYMFIQTQDTPNPNSLKFIPGKPVLETRTMDFPTPAAAFRSPLA RQLFRIEGVKSVFFGPDFITVTKENEELDWNLLKPDIYATIMDFFASGLP LVTEETPSGEAGSEEDDEVVAMIKELLDTRIRPTVQEDGGDVIYKGFEDG IVQLKLQGSCTSCPSSIITLKNGIQNMLQFYIPEVEGVEQVMDDESDEKE ANSP
    • Amino acids
      1 to 254
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab162125 in the following tested applications.

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

  • Applications

    Western blot

    ELISA

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 mg/ml.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

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

  • ab162125 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab162125 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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