Overview

  • Product name

    Recombinant Human NUBPL protein
  • Protein length

    Full length protein

Description

  • Nature

    Recombinant
  • Source

    Wheat germ
  • Amino Acid Sequence
    • Species

      Human
    • Sequence

      MVCGRQLSGAGSETLKQRRTQIMSRGLPKQKPIEGVKQVIVVASGKGGVG KSTTAVNLALALAANDSSKAIGLLDVDVYGPSVPKMMNLKGNPELSQSNL MRPLLNYGIACMSMGFLVEESEPVVWRGLMVMSAIEKLLRQVDWGQLDYL VVDMPPGTGDVQLSVSQTIPITGAVIVSTPQDIALMDAHKGAEMFRRVHV PVLGLVQNMSVFQCPKCKHKTHIFGADGARKLAQTLGLEVLGDIPLHLNI REASDTGQPIVFSQPESDEAKAYLRIAVEVVRRLPSPSE
    • Amino acids

      1 to 289
    • Tags

      GST tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab164228 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.

    Previously labelled as Nucleotide binding protein like.

  • 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

    • C14orf127
    • FLJ12660
    • huInd1
    • IND1 homolog
    • Iron-sulfur protein NUBPL
    • Nubpl
    • NUBPL_HUMAN
    • nucleotide binding protein like
    • Nucleotide-binding protein-like
    see all
  • Function

    Required for the assembly of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I). May deliver of one or more Fe-S clusters to complex I subunits.
  • Tissue specificity

    Highest expression in liver and kidney. expressed at significant levels in small intestine and brain (at protein level).
  • Involvement in disease

    Defects in NUBPL are a cause of mitochondrial complex I deficiency (MT-C1D) [MIM:252010]. A disorder of the mitochondrial respiratory chain that causes a wide range of clinical manifestations from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease.
  • Sequence similarities

    Belongs to the Mrp/NBP35 ATP-binding proteins family.
  • Cellular localization

    Mitochondrion.
  • Information by UniProt

Images

  • ab164228 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab164228 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab164228.
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