Anti-HLCS antibody (ab100925)
- Product nameAnti-HLCS antibodySee all HLCS primary antibodies ...
- DescriptionRabbit polyclonal to HLCS
- Tested applicationsWB, ELISA, IHC-P more details
- Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat
Recombinant fragment, corresponding to amino acids 544-725 of Human HLCS (BC060787).
- Positive controlHuman Fetal Lung, Human Fetal Kidney, Human Fetal Skeletal Muscle
- Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
- Storage bufferPreservative: 0.02% Sodium Azide
Constituents: PBS, pH 7.2
- Concentration information loading...
- PurityProtein A purified
- Clonality Polyclonal
- Research Areas
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||WB: 1/500 - 1/1000. Predicted molecular weight: 81 kDa.|
|ELISA||ELISA: 1/20000 - 1/80000.|
|IHC-P||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.
- Biotin [acetyl CoA carboxylase] ligase antibodyBiotin [methylcrotonoyl CoA carboxylase] ligase antibodyBiotin [methylmalonyl CoA carboxytransferase] ligase antibody
- Biotin [propionyl CoA carboxylase [ATP hydrolyzing]] ligase antibodyBiotin apo protein ligase antibodyBiotin apo-protein ligase antibodyBiotin protein ligase antibodyBiotin--[acetyl-CoA-carboxylase] ligase antibodyBPL1_HUMAN antibodyEC 6.3.4.- antibodyHCS antibodyHLCS antibodyHolocarboxylase synthetase antibodyHolocarboxylase synthetase, EC 6.3.4 antibody
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.