Description

  • Product name

    Recombinant Human PCCB protein
  • Expression system

    Escherichia coli
  • Accession

  • Protein length

    Protein fragment
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Predicted molecular weight

      28 kDa
    • Amino acids

      7 to 266

Specifications

Our Abpromise guarantee covers the use of ab127093 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

  • Form

    Lyophilised
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

    Constituents: 0.32% Tris HCl, 0.58% Sodium chloride

  • Reconstitution
    Reconstitute with water to desired concentration.

General Info

  • Alternative names

    • DKFZp451E113
    • PCCase subunit beta
    • pccB
    • PCCB_HUMAN
    • pccBC Complementation group
    • Propanoyl CoA:carbon dioxide ligase subunit beta
    • Propanoyl-CoA:carbon dioxide ligase subunit beta
    • Propionyl CoA carboxylase beta chain, mitochondrial
    • propionyl Coenzyme A carboxylase, beta polypeptide
    • Propionyl-CoA carboxylase , beta subunit
    • Propionyl-CoA carboxylase beta chain, mitochondrial
    • R74805
    see all
  • Pathway

    Metabolic intermediate metabolism; propanoyl-CoA degradation; succinyl-CoA from propanoyl-CoA: step 1/3.
  • Involvement in disease

    Defects in PCCB are the cause of propionic acidemia type II (PA-2) [MIM:606054]. PA-2 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein.
  • Sequence similarities

    Belongs to the AccD/PCCB family.
    Contains 1 carboxyltransferase domain.
  • Cellular localization

    Mitochondrion matrix.
  • Information by UniProt

References

ab127093 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab127093.
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