Overview

Description

  • Nature

    Recombinant
  • Source

    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Sequence

      MEDRLHMDNGLVPQKIVSVHLQDSTLKEVKDQVSNKQAQILEPKPEPSLE IKPEQDGMEHVGRDDPKALGEEPKQRRGSASGSEPAGDSDRGGGPVEHYH LHLSSCHECLELENSTIESVKFASAENIPDLPYDYSSSLESVADETSPER EGRRVNLTGKAPNILLYVGSDSQEALGRFHEVRSVLADCVDIDSYILYHL LEDSALRDPWTDNCLLLVIATRESIPEDLYQKFMAYLSQGGKVLGLSSSF TFGGFQVTSKGALHKTVQNLVFSKADQSEVKLSVLSSGCRYQEGPVRLSP GRLQGHLENEDKDRMIVHVPFGTRGGEAVLCQVHLELPPSSNIVQTPEDF NLLKSSNFRRYEVLREILTTLGLSCDMKQVPALTPLYLLSAAEEIRDPLM QWLGKHVDSEGEIKSGQLSLRFVSSYVSEVEITPSCIPVVTNMEAFSSEH FNLEIYRQNLQTKQLGKVILFAEVTPTTMRLLDGLMFQTPQEMGLIVIAA RQTEGKGRGGNVWLSPVGCALSTLLISIPLRSQLGQRIPFVQHLMSVAVV EAVRSIPEYQDINLRVKWPNDIYYSDLMKIGGVLVNSTLMGETFYILIGC GFNVTNSNPTICINDLITEYNKQHKAELKPLRADYLIARVVTVLEKLIKE FQDKGPNSVLPLYYRYWVHSGQQVHLGSAEGPKVSIVGLDDSGFLQVHQE GGEVVTVHPDGNSFDMLRNLILPKRR
    • Molecular weight

      107 kDa including tags
    • Amino acids

      1 to 726

Associated products

Specifications

Our Abpromise guarantee covers the use of ab132809 in the following tested applications.

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

  • Applications

    ELISA

    SDS-PAGE

    Western blot

  • Form

    Liquid
  • Additional notes

    Protein concentration is above or equal to 0.05 µg/µl.
  • 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

    • 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

Images

  • 12.5% SDS-PAGE analysis of ab132809 stained with Coomassie Blue.

References

ab132809 has not yet been referenced specifically in any publications.

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