Overview

Description

  • Nature

    Recombinant
  • Source

    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Sequence

      GSPEFHMGKP LLGGPFSLTT HTGERKTDKD YLGQWLLIYF GFTHCPDVCP EELEKMIQVV DEIDSITTLP DLTPLFISID PERDTKEAIA NYVKEFSPKL VGLTGTREEV DQVARAYRVY YSPGPKDEDE DYIVDHTIIM YLIGPDGEFL DYFGQNKRKG EIAASIATHM RPYRKKS
    • Molecular weight

      19 kDa
    • Amino acids

      133 to 300

Specifications

Our Abpromise guarantee covers the use of ab151389 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

  • Endotoxin level

    < 1.000 Eu/µg
  • Purity

    > 95 % SDS-PAGE.
    Greater than 95% as determined by reducing SDS-PAGE.
  • Form

    Lyophilised
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. The lyophilized protein is stable for a few weeks at room temperature. Store at -20°C long term.

    pH: 7.20
    Constituents: 99% Phosphate Buffer, 0.02% DTT

  • Reconstitution
    Lyophilized from a 0.2 µM filtered solution. Always centrifuge tubes before opening. Do not mix by vortex or pipetting. Dissolve the lyophilized protein in 1X PBS. It is not recommended to reconstitute to a concentration less than 100 µg/ml.
    Reconstituted protein solution can be stored at 4-7°C for 2-7 days. Aliquots of reconstituted samples are stable at < -20°C for 3 months.

General Info

  • Alternative names

    • Cytochrome oxidase deficient homolog
    • Cytochrome oxidase deficient homolog 1
    • Protein SCO1 homolog mitochondrial
    • Protein SCO1 homolog, mitochondrial
    • SCO (cytochrome oxidase deficient yeast) homolog 1
    • SCO cytochrome oxidase deficient homolog 1
    • SCO cytochrome oxidase deficient homolog 1 (yeast)
    • sco1
    • SCO1_HUMAN
    • SCOD1
    see all
  • Function

    Thought to play a role in cellular copper homeostasis, mitochondrial redox signaling or insertion of copper into the active site of COX.
  • Tissue specificity

    Predominantly expressed in tissues characterized by high rates of oxidative phosphorylation (OxPhos), including muscle, heart, and brain.
  • Involvement in disease

    Defects in SCO1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]; also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome.
  • Sequence similarities

    Belongs to the SCO1/2 family.
  • Cellular localization

    Mitochondrion.
  • Information by UniProt

References

ab151389 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab151389.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
For licensing inquiries, please contact partnerships@abcam.com

Sign up