Anti-HLCS antibody (ab100925)
Key features and details
- Rabbit polyclonal to HLCS
- Suitable for: WB, IHC-P
- Reacts with: Human
- Isotype: IgG
Overview
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Product name
Anti-HLCS antibody -
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).
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Positive control
- Human Fetal Lung, Human Fetal Kidney, Human Fetal Skeletal Muscle
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General notes
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
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Properties
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Form
Lyophilized: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 instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles. -
Storage buffer
Constituent: PBS -
Concentration information loading...
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Purity
Protein A purified -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
Applications
The Abpromise guarantee
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 |
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WB |
1/500 - 1/1000. Predicted molecular weight: 81 kDa.
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IHC-P |
1/100 - 1/500.
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Notes |
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WB
1/500 - 1/1000. Predicted molecular weight: 81 kDa. |
IHC-P
1/100 - 1/500. |
Target
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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
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Database links
- Entrez Gene: 3141 Human
- Entrez Gene: 110948 Mouse
- Entrez Gene: 288240 Rat
- Omim: 609018 Human
- SwissProt: P50747 Human
- SwissProt: Q920N2 Mouse
- Unigene: 371350 Human
- Unigene: 30921 Mouse
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Alternative names
- Biotin [acetyl CoA carboxylase] ligase antibody
- Biotin [methylcrotonoyl CoA carboxylase] ligase antibody
- Biotin [methylmalonyl CoA carboxytransferase] ligase antibody
see all
Images
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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.
Protocols
Datasheets and documents
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SDS download
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Datasheet download
References (0)
ab100925 has not yet been referenced specifically in any publications.