Recombinant Human Coproporphyrinogen Oxidase (CPOX) protein (ab158194)

Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      SCARAVVPSYIPLVKKHCDDSFTPQEKLWQQLRRGRYVEFNLLYDRGTKF GLFTPGSRIESILMSLPLTARWEYMHSPSENSKEAEILEVLRHPRDWV
    • Amino acids
      356 to 453
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab158194 in the following tested applications.

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

  • Applications

    ELISA

    Western blot

  • 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
    • Coprogen oxidase
    • COPROPORPHYRIA
    • Coproporphyrinogen III oxidase
    • Coproporphyrinogen-III oxidase
    • Coproporphyrinogenase
    • COX
    • CPO
    • Cpox
    • CPX
    • HEM 6
    • Hem-6
    • Hem6
    • HEM6_HUMAN
    • mitochondrial
    see all
  • Function
    Key enzyme in heme biosynthesis. Catalyzes the oxidative decarboxylation of propionic acid side chains of rings A and B of coproporphyrinogen III.
  • Pathway
    Porphyrin metabolism; protoporphyrin-IX biosynthesis; protoporphyrinogen-IX from coproporphyrinogen-III (O2 route): step 1/1.
  • Involvement in disease
    Defects in CPOX are the cause of hereditary coproporphyria (HCP) [MIM:121300]. HCP is an acute hepatic porphyria and an autosomal dominant disease characterized by neuropsychiatric disturbances and skin photosensitivity. Biochemically, there is an overexcretion of coproporphyrin III in the urine and in the feces. HCP is clinically characterized by attacks of abdominal pain, neurological disturbances, and psychiatric symptoms. The symptoms are generally manifested with rapid onset, and can be precipitated by drugs, alcohol, caloric deprivation, infection, endocrine factors or stress. A severe variant form is harderoporphyria, which is characterized by earlier onset attacks, massive excretion of harderoporphyrin in the feces, and a marked decrease of coproporphyrinogen IX oxidase activity.
  • Sequence similarities
    Belongs to the aerobic coproporphyrinogen-III oxidase family.
  • Cellular localization
    Mitochondrion intermembrane space.
  • Information by UniProt

Images

  • ab158194 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab158194 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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