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Huntingtin Antibody
SPA-05362
| Size | Price |
| 25 µg | Online Inquiry |
| 100 µg | Online Inquiry |
| More Options | Online Inquiry |
| Target Information | |
|---|---|
| Target Name | Huntingtin |
| Gene Abbr. | HTT |
| Gene ID | 3064 |
| Full Name | huntingtin |
| Alias | HD, IT15, LOMARS |
| Introduction | Huntington's Disease (HD) is a fatal neurodegenerative disorder characterized by psychiatric, cognitive, and motor dysfunction. Neuropathology of HD involves specific neuronal subpopulations: GABA-ergic neurons of the striatum and neurons within the cerebral cortex selectively degenerate. The genetic analysis of HD has been the flagship study of inherited neurological diseases from initial chromosomal localization to identification of the gene.Huntingtin is a large (340-350 kD) cytosolic protein that may be involved in a number of cellular functions such as transcription, gastrulation, neurogenesis, neurotransmission, axonal transport, neural positioning, and apoptosis. The HD gene from unaffected individuals contains between 6 and 34 CAG trinucleotide repeats, with expansion beyond this range causing the onset of disease symptoms. A strong inverse correlation exists between the age of onset in patients and the number of huntingtin gene CAG repeats encoding a stretch of polyglutamine peptides. The huntingtin protein undergoes numerous post-translational modifications including phosphorylation, ubiquitination, sumoylation, palmitoylation, and cleavage. Phosphorylation of Ser421 by Akt can partially counteract the toxicity that results from the expanded polyglutamine tract. Varying Akt expression in the brain correlates with regional differences in huntingtin protein phosphorylation; this pattern inversely correlates with the regions that are most affected by degeneration in diseased brain. A key step in the disease is the proteolytic cleavage of huntingtin protein into amino-terminal fragments that contain expanded glutamine repeats and translocate into the nucleus. Caspase mediated cleavage of huntingtin at Asp513 is associated with increased polyglutamine aggregate formation and toxicity. Phosphorylation of Ser434 by CDK5 protects against cleavage. |
| Product Details | |
|---|---|
| Host | Mouse |
| Clonality | Monoclonal |
| Clone No. | HDB4E10 |
| Isotype | IgG1 |
| Immunogen | Recombinant fragment, corresponding to amino acids 1844-2131 of Huntingtin. |
| Usage | |
|---|---|
| Application | WB, IHC, IP |
| Dilutions | Western Blot (1:100-1:2000); Immunohistochemistry (1:10-1:500); Immunoprecipitation (1:10-1:500) |
| MW(KDa) | 350 |
| Reactivity | Human, Mouse, Rabbit |
| Specificity | This reacts with an epitope corresponding to the HDB region (amino acids 1844 - 2131) of the huntingtin protein. |
| Storage & Handling | |
|---|---|
| Storage Buffer | PBS. |
| Preservative | 0.09% Sodium Azide |
| Storage Temp. | Store at 4 °C for a short term. Aliquot and store at -20 °C for long-term storage. |
| Handling | Avoid freeze-thaw cycles. |
For research use only. Not intended for any clinical use. No products from CD BioSciences may be resold, modified for resale or used to manufacture commercial products without prior written approval from CD BioSciences.