Overview

Description

  • Nature

    Recombinant
  • Source

    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Molecular weight

      21 kDa
    • Amino acids

      544 to 725
    • Tags

      His-DHFR tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab127602 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

    • Biotin [acetyl CoA carboxylase] ligase
    • Biotin [methylcrotonoyl CoA carboxylase] ligase
    • Biotin [methylmalonyl CoA carboxytransferase] ligase
    • Biotin [propionyl CoA carboxylase [ATP hydrolyzing]] ligase
    • Biotin apo protein ligase
    • Biotin apo-protein ligase
    • Biotin protein ligase
    • Biotin--[acetyl-CoA-carboxylase] ligase
    • BPL1_HUMAN
    • HCS
    • HLCS
    • Holocarboxylase synthetase
    • Holocarboxylase synthetase, EC 6.3.4
    see all
  • Function

    Post-translational modification of specific protein by attachment of biotin. Acts on various carboxylases such as acetyl-CoA-carboxylase, pyruvate carboxylase, propionyl CoA carboxylase, and 3-methylcrotonyl CoA carboxylase.
  • Tissue specificity

    Mostly expressed in muscle, placenta, in lesser extent in the brain, kidney, pancreas, liver and lung.
  • Involvement in disease

    Defects in HLCS are the cause of holocarboxylase synthetase deficiency (HLCS deficiency) [MIM:253270]; also known as biotin-responsive multiple carboxylase deficiency. HLCS deficiency is a neonatal form of multiple carboxylase deficiency, an autosomal recessive disorder characterized by metabolic ketoacidosis, hyperammonemia, excretion of abnormal organic acid metabolites and dermatitis. Clinical and biochemical symptoms improve dramatically with administration of biotin.
  • Sequence similarities

    Belongs to the biotin--protein ligase family.
  • Cellular localization

    Cytoplasm. Mitochondrion.
  • Information by UniProt

References

ab127602 has not yet been referenced specifically in any publications.

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