Anti-Huntingtin antibody [2401C1a] (ab67092)

Overview

• Product nameAnti-Huntingtin antibody [2401C1a]See all Huntingtin primary antibodies ...
• Description
  Mouse monoclonal [2401C1a] to Huntingtin
• Tested applicationsWB, Dot Blot more details
• Species reactivity
  Reacts with: Human
  
• Immunogen

  Recombinant fragment (Human) from an internal region of Huntingtin.

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: 0.05% Sodium Azide
  Constituents: 1% BSA, PBS (8mM Dibasic monohydrogen sodium phosphate, 3mM Potassium chloride, 140mM Sodium chloride, 1.5mM Monobasic dihydrogen sodium phosphate), pH 7.4
• Concentration information loading...
• PurityProtein G purified
• Purification notesThis antibody was purified using protein G column chromatography from culture supernatant of hybridoma cultured in a medium containing bovine IgG depleted (approximately 95%) fetal bovine serum, and filtered through a 0.22µm membrane.
• Clonality Monoclonal
• Clone number2401C1a
• IsotypeIgG2b
• 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
  • Omim: 143100 Human
  • Omim: 613004 Human
  • SwissProt: P42858 Human
  • Unigene: 518450 Human

• 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

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Anti-Huntingtin antibody [2401C1a] images

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  Western blot - Huntingtin antibody [2401C1a] (ab67092)
  Anti-Huntingtin antibody [2401C1a] (ab67092) + immunising recombinant fragment
  
  Observed band size : 35 kDa (why is the actual band size different from the predicted?)