Key features and details
- Rabbit polyclonal to HLCS
- Suitable for: WB, IHC-P
- Reacts with: Human
- Isotype: IgG
Product nameAnti-HLCS antibody
See all HLCS primary antibodies
DescriptionRabbit polyclonal to HLCS
Tested applicationsSuitable for: WB, IHC-Pmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat
Recombinant fragment, corresponding to amino acids 544-725 of Human HLCS (BC060787).
- Human Fetal Lung, Human Fetal Kidney, Human Fetal Skeletal Muscle
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In preparation for this, we have started to update the applications & species that this product is Abpromise guaranteed for.
We are also updating the applications & species that this product has been “predicted to work with,” however this information is not covered by our Abpromise guarantee.
Applications & species from publications and Abreviews that have not been tested in our own labs or in those of our suppliers are not covered by the Abpromise guarantee.
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FormLyophilized:Reconstitute with 200ul distilled sterile water. Please note that if you receive this product in liquid form it has already been reconstituted as described and no further reconstitution is necessary.
Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Storage bufferConstituent: PBS
Concentration information loading...
PurityProtein A purified
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.
|WB||1/500 - 1/1000. Predicted molecular weight: 81 kDa.|
|IHC-P||1/100 - 1/500.|
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
- Biotin [acetyl CoA carboxylase] ligase antibody
- Biotin [methylcrotonoyl CoA carboxylase] ligase antibody
- Biotin [methylmalonyl CoA carboxytransferase] ligase antibody
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.
ab100925 has not yet been referenced specifically in any publications.