Anti-Huntingtin antibody [1A771] (ab13583)
- Product nameAnti-Huntingtin antibody [1A771]See all Huntingtin primary antibodies ...
- DescriptionMouse monoclonal [1A771] to Huntingtin
- Tested applicationsWB more details
- Species reactivityReacts with: Mouse, Human
The antibody was raised against a fusion protein containing 62 glutamine amino acid repeats.
- Positive controlLymphoblast cell line extracts.
- 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
- Concentration information loading...
- PurityProtein G purified
- Clonality Monoclonal
- Clone number1A771
- Research Areas
Our Abpromise guarantee covers the use of ab13583 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||WB: Use a concentration of 1 µg/ml. Predicted molecular weight: 377 kDa.|
- 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.
- 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
References for Anti-Huntingtin antibody [1A771] (ab13583)
ab13583 has not yet been referenced specifically in any publications.