Recombinant Human Cytochrome C Oxidase subunit VIb protein (ab114917)

Overview

Description

  • NatureRecombinant
  • SourceWheat germ
  • Amino Acid Sequence
    • AccessionP14854
    • SpeciesHuman
    • SequenceMAEDMETKIKNYKTAPFDSRFPNQNQTRNCWQNYLDFHRCQKAMTAKGGD ISVCEWYQRVYQSLCPTSWVTDWDEQRAEGTFPGKI
    • Molecular weight35 kDa including tags
    • Amino acids1 to 87

Specifications

Our Abpromise guarantee covers the use of ab114917 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

    SDS-PAGE

    ELISA

  • FormLiquid
  • Additional notesProtein concentration is above or equal to 0.05 mg/ml. Best use within three months from the date of receipt of this protein.
  • 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: 0.3% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names
    • COX 6B
    • COX VIb 1
    • COX VIb-1
    • COX6B
    • COX6B1
    • COXG
    • COXVIb1
    • CX6B1_HUMAN
    • Cytochrome c oxidase subunit 6B1
    • Cytochrome c oxidase subunit VIb
    • Cytochrome c oxidase subunit VIb isoform 1
    • Cytochrome c oxidase subunit Vib polypeptide 1
    • cytochrome c oxidase subunit VIb polypeptide 1 (ubiquitous)
    see all
  • FunctionConnects the two COX monomers into the physiological dimeric form.
  • Involvement in diseaseDefects in COX6B1 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 similaritiesBelongs to the cytochrome c oxidase subunit 6B family.
  • Cellular localizationMitochondrion intermembrane space.
  • Information by UniProt

Recombinant Human Cytochrome C Oxidase subunit VIb protein images

  • 12.5% SDS-PAGE showing ab114917 at approximately 35.20kDa.
    Stained with Coomassie Blue.

References for Recombinant Human Cytochrome C Oxidase subunit VIb protein (ab114917)

ab114917 has not yet been referenced specifically in any publications.

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