Overview

  • Product name

  • Description

    Rabbit polyclonal to HLCS
  • Host species

    Rabbit
  • Tested applications

    Suitable 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

  • 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
  • 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

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

ab100925 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

Question
Answer

Thank you for confirming these details and for your cooperation. The details provided enable us to closely monitor the quality of our products.

I am sorry these antibodies (anti-HLCS and anti-HMGCS1) did not perform as stated on the datasheet and for the inconvenience this has caused. As requested, I have issued free of charge replacements with the order number ****.

To check the status of the order please contact our Customer Service team and reference this number.

Please note that these free of charge replacement vials are also covered by our Abpromise guarantee. Should you still be experiencing difficulties, or if you have any further questions, please do not hesitate to let us know.

I wish you the best of luck with your research.

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