Anti-Huntingtin antibody [1A771] (ab13583)

Overview

• Product nameAnti-Huntingtin antibody [1A771]See all Huntingtin primary antibodies ...
• Description
  Mouse monoclonal [1A771] to Huntingtin
• Tested applicationsWB more details
• Species reactivity
  Reacts with: Mouse, Human
  
• Immunogen

  The antibody was raised against a fusion protein containing 62 glutamine amino acid repeats.

• Positive controlLymphoblast cell line extracts.

Properties

• FormLiquid
• Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
• Storage bufferPreservative: 0.02% Sodium Azide
  Constituents: PBS
• Concentration information loading...
• PurityProtein G purified
• Clonality Monoclonal
• Clone number1A771
• IsotypeIgG1
• Research Areas
  • Neuroscience
  • Neurology process
  • Neurodegenerative disease
  • Huntington's disease

Applications

Target

• FunctionMay play a role in microtubule-mediated transport or vesicle function.
• Tissue specificityExpressed in the brain cortex (at protein level). Widely expressed with the highest level of expression in the brain (nerve fibers, varicosities, and nerve endings). In the brain, the regions where it can be mainly found are the cerebellar cortex, the neocortex, the striatum, and the hippocampal formation.
• Involvement in diseaseDefects in HTT are the cause of Huntington disease (HD) [MIM:143100]. HD is an autosomal dominant neurodegenerative disorder characterized by involuntary movements (chorea), general motor impairment, psychiatric disorders and dementia. Onset of the disease occurs usually in the third or fourth decade of life and symptoms progressively worsen leading to death in 10 to 20 years. Onset and clinical course depend on the degree of poly-Gln repeat expansion, longer expansions resulting in earlier onset and more severe clinical manifestations. HD affects 1 in 10,000 individuals of European origin. Neuropathology of Huntington disease displays a distinctive pattern with loss of neurons, especially in the caudate and putamen (striatum).
• Sequence similaritiesBelongs to the huntingtin family.
  Contains 10 HEAT repeats.
• DomainThe N-terminal Gln-rich and Pro-rich domain has great conformational flexibility and is likely to exist in a fluctuating equilibrium of alpha-helical, random coil, and extended conformations.
• Post-translational
  modificationsCleaved by apopain downstream of the polyglutamine stretch. The resulting N-terminal fragment is cytotoxic and provokes apoptosis.
  Forms with expanded polyglutamine expansion are specifically ubiquitinated by SYVN1, which promotes their proteasomal degradation.
• Cellular localizationCytoplasm. Nucleus. The mutant Huntingtin protein colocalizes with AKAP8L in the nuclear matrix of Huntington's disease neurons.

Target information above from: UniProt accession P42858 The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010) .

Information by UniProt
• Database links
  • Entrez Gene: 3064 Human
  • Entrez Gene: 15194 Mouse
  • Omim: 143100 Human
  • Omim: 613004 Human
  • SwissProt: P42858 Human
  • SwissProt: P42859 Mouse
  • Unigene: 518450 Human
  • Unigene: 209071 Mouse

  • Unigene: 482929 Mouse

  see all

• Alternative names
  AI256365 antibodyC430023I11Rik antibodyHD antibody

  HD protein antibodyHD_HUMAN antibodyHDH antibodyHTT antibodyHuntingtin antibodyHUNTINGTON CHOREA antibodyHuntington disease protein antibodyHuntington's disease protein homolog antibodyIT 15 antibodyIT15 antibodyOTTMUSP00000026909 antibodyZHD antibody

  see all

Anti-Huntingtin antibody [1A771] images

• 

  Western blot - Huntingtin antibody [1A771] (ab13583)
  
  
  Predicted band size : 377 kDa