Overview

  • Product nameAnti-HLCS antibody
    See all HLCS primary antibodies
  • Description
    Rabbit polyclonal to HLCS
  • Tested applicationsSuitable for: WB, IHC-Pmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse, Rat
  • Immunogen

    Recombinant fragment, corresponding to amino acids 544-725 of Human HLCS (BC060787).

  • Positive control
    • Human Fetal Lung, Human Fetal Kidney, Human Fetal Skeletal Muscle

Properties

Applications

Our Abpromise guarantee covers the use of ab100925 in the following tested applications.

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

Application Abreviews Notes
WB 1/500 - 1/1000. Predicted molecular weight: 81 kDa.
IHC-P 1/100 - 1/500.

Target

  • FunctionPost-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 specificityMostly expressed in muscle, placenta, in lesser extent in the brain, kidney, pancreas, liver and lung.
  • Involvement in diseaseDefects 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 similaritiesBelongs to the biotin--protein ligase family.
  • Cellular localizationCytoplasm. Mitochondrion.
  • Information by UniProt
  • Database links
  • Alternative names
    • Biotin [acetyl CoA carboxylase] ligase antibody
    • Biotin [methylcrotonoyl CoA carboxylase] ligase antibody
    • Biotin [methylmalonyl CoA carboxytransferase] ligase antibody
    • Biotin [propionyl CoA carboxylase [ATP hydrolyzing]] ligase antibody
    • Biotin apo protein ligase antibody
    • Biotin apo-protein ligase antibody
    • Biotin protein ligase antibody
    • Biotin--[acetyl-CoA-carboxylase] ligase antibody
    • BPL1_HUMAN antibody
    • HCS antibody
    • HLCS antibody
    • Holocarboxylase synthetase antibody
    • Holocarboxylase synthetase, EC 6.3.4 antibody
    see all

Anti-HLCS antibody images

  • All lanes : Anti-HLCS antibody (ab100925) at 1/500 dilution

    Lane 1 : Human fetal lung lysate
    Lane 2 : Human fetal kidney lysate


    Predicted band size : 81 kDa
  • Cytoplasmic staining of HLCS in a Formalin/PFA-fixed paraffin-embedded section of Human Fetal Skeletal Muscle using ab100925 at a dilution of 1/100.

References for Anti-HLCS antibody (ab100925)

ab100925 has not yet been referenced specifically in any publications.

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