Anti-Huntingtin

Description

• General description: Huntingtin (UniProt: P42858; also known as Huntington disease protein, HD protein) is encoded by the HTT (also known as HD, IT15) gene (Gene ID: 3064) in human. The protein is found in the perinuclear region along with microtubules, and in the centrosomal region along with gamma-tubulin. It is expressed in the brain and is mainly found in the cerebellar cortex, the neocortex, the striatum, and the hippocampal formation. Huntingtin is necessary for neuronal survival and is involved in synaptic vesicle trafficking, microtubule binding and may also have a role in apoptosis. It plays a role in microtubule-mediated transport and vesicle function. Huntingtin protein contains a nuclear export signal (aa 2395-2404) and 5 HEAT (Huntington, Elongation Factor 3, PR65/A, TOR) domains. Huntingtin protein is cleaved by apopain downstream of the polyglutamine stretch and the resulting N-terminal fragment is cytotoxic and provokes apoptosis. Phosphorylation at Serine 1179 and 1199 by CDK5 in response to DNA damage in nuclei of neurons protects neurons against polyglutamine expansion as well as DNA damage mediated toxicity. Mutations in HTT gene cause Huntington disease that is characterized by involuntary movements (chorea), general motor impairment, psychiatric disorders and dementia. Wild-type huntingtin and anti-huntingtin antibody reduces aggregation and cellular toxicity of the mutant huntingtin form in mammalian cell models of Huntington disease.
• Specificity: This rabbit polyclonal antibody detects Huntington disease protein in human and mouse brain. It targets an epitope within 180 amino acids from the N-terminal region.
• Immunogen: Epitope: N-terminus
• Application: Anti-Huntingtin, Cat. No. ABN903, is a highly specific rabbit polyclonal antibody that targets Huntington Disease Protein and has been tested for use in Immunohistochemistry (Paraffin) and Western Blotting.
• Quality: Evaluated by Immunohistochemistry in human cerebral cortex and human huntington′s diseased brain tissues.Immunohistochemistry Analysis: A 1:50-250 dilution of this antibody detected Huntingtin in human cerebral cortex and human Huntington′s diseased brain tissues.
• Target description: 347.60 kDa calculated.
• Physical form: Affinity Purified
• Storage and Stability: Stable for 1 year at 2-8°C from date of receipt.
• Other Notes: Concentration: Please refer to lot specific datasheet.
• Disclaimer: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.