Anti-Huntingtin antibody (ab76214)
- Product nameAnti-Huntingtin antibodySee all Huntingtin primary antibodies ...
- DescriptionRabbit polyclonal to Huntingtin
- Tested applicationsWB, IHC-Fr, IHC-P more details
- Species reactivityReacts with: Mouse, Rat
Predicted to work with: Human
Synthetic peptide from an internal sequence of Rat Huntingtin, conjugated to an immunogenic carrier protein
- Storage instructionsStore at +4°C short term (1-2 weeks). Add glycerol to a final volume of 50%, aliquot and store at -20°C. Avoid repeated freeze / thaw cycles.
- Storage bufferPreservative: None
Constituents: 1X PBS
- Concentration information loading...
- PurityIgG fraction
- Clonality Polyclonal
- Research Areas
Our Abpromise guarantee covers the use of ab76214 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
IHC-Fr: Use at a concentration of 10 - 50 µg/ml.
WB: Use at a concentration of 10 - 50 µg/ml. Predicted molecular weight: 344 kDa.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
- 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.
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.
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References for Anti-Huntingtin antibody (ab76214)
ab76214 has not yet been referenced specifically in any publications.