Anti-HLCS antibody (ab100925)

Overview

• Product nameAnti-HLCS antibodySee all HLCS primary antibodies ...
• Description
  Rabbit polyclonal to HLCS
• Tested applicationsWB, ELISA, IHC-P more 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 controlHuman Fetal Lung, Human Fetal Kidney, Human Fetal Skeletal Muscle

Properties

• FormLiquid
• 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
• IsotypeIgG
• Research Areas
  • Metabolism
  • Pathways and Processes
  • Mitochondrial Metabolism
  • Mitochondrial markers

  • Metabolism
  • Types of disease
  • Cancer

  • Signal Transduction
  • Metabolism
  • Mitochondrial

Applications

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.

Target information above from: UniProt accession P50747 The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010) .

Information by UniProt
• 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

• Alternative names
  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

  see all

Anti-HLCS antibody images

• 

  Western blot - HLCS antibody (ab100925)
  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
  
• 

  Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - HLCS antibody (ab100925)
  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.