| Overview |
| bsm-70519M |
| Huntingtin, mutant-specific Antibody |
| WB |
| This antibody detects expanded polyQ repeat form of Htt in western blot, and does not detect normal Htt in a lymphoblast cell line from HD patients. |
| Human, Mouse |
| Specifications |
| Unconjugated |
| Mouse |
| Clone 1A771 was generated from Huntingtin mutant protein containing 62 glutamine amino acid repeats. |
| Monoclonal |
| 1A771 |
| IgG1 |
| Purified by Protein G. |
| PBS + 0.2% gelatin and 0.05% NaN3. |
| Recommended that the undiluted antibody be aliquoted into smaller working volumes (10-30 uL/vial depending on usage) upon arrival and stored long term at -20° C or -80° C, while keeping a working aliquot stored at 4° C for short term. Avoid freeze/thaw cycles. Stable for at least 1 year. |
| Target |
| P25963 |
| deltaNp73 |
| Huntington's disease (HD) is a neurodegenerative disorder caused by an expanding polyglutamine repeat in the huntingtin (Htt) gene. HD is a mid-life onset autosomal dominant neurodegenerative disease that is characterized by psychiatric disorders, dementia, and involuntary movements (chorea), leading to death in 10-20 years. The HD gene product HTT is widely expressed in human tissues, with the highest level of expression in the brain. Furthermore, HTT is expressed at similar levels in HD patients and controls, which suggests that the expansion of the polyglutamine repeat induces a toxic gain of function perhaps through interactions with other cellular proteins. Using a yeast two-hybrid approach, HAP1 (huntington associated protein 1) was identified. HAP1 associates with HTT. The in vitro data suggest that the association between HAP1 and HTT is enhanced by increasing length of the glutamine repeat in HTT. |
| Application Dilution |
| WB |
1:300-5000 |
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