Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      SIDPERDTKEAIANYVKEFSPKLVGLTGTREEVDQVARAYRVYYSPGPKD EDEDYIVDHTIIMYLIGPDGEFLDYFGQNKRKGEIAASIATHMRPYRKKS
    • Amino acids
      202 to 301
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab159485 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
    • 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

Images

  • ab159485 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab159485 has not yet been referenced specifically in any publications.

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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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