Overview

  • Product name

    Recombinant Human PCB protein
  • Protein length

    Protein fragment

Description

  • Nature

    Recombinant
  • Source

    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Molecular weight

      33 kDa
    • Amino acids

      74 to 371

Specifications

Our Abpromise guarantee covers the use of ab127137 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. Store at -20ºC.

    Constituents: 0.32% Tris HCl, 0.58% Sodium chloride

  • Reconstitution
    Reconstitute with water to desired concentration.

General Info

  • Alternative names

    • mitochondrial
    • OTTHUMP00000235155
    • OTTHUMP00000235156
    • PC
    • PCB
    • Pcx
    • PYC_HUMAN
    • Pyruvate carboxylase
    • Pyruvate carboxylase mitochondrial
    • Pyruvic carboxylase
    see all
  • Function

    Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate.
  • Pathway

    Carbohydrate biosynthesis; gluconeogenesis.
  • Involvement in disease

    Defects in PC are the cause of pyruvate carboxylase deficiency (PC deficiency) [MIM:266150]. PC deficiency leads to lactic acidosis, mental retardation and death. It occurs in three forms: mild or type A, severe neonatal or type B, and a very mild lacticacidemia.
  • Sequence similarities

    Contains 1 ATP-grasp domain.
    Contains 1 biotin carboxylation domain.
    Contains 1 biotinyl-binding domain.
    Contains 1 carboxyltransferase domain.
  • Cellular localization

    Mitochondrion matrix.
  • Information by UniProt

References

ab127137 has not yet been referenced specifically in any publications.

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