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Anti -Huntingtin Protein (Huntington Disease, Htt)
Anti -Huntingtin Protein (Huntington Disease, Htt)
Pricing
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| Clone |
Host |
Grade |
Applications |
| Monoclonal |
Rabbit |
Supernatant |
B IH IC FC |
|
| Huntingtin protein (Htt) is a 348kD protein product of Huntington’s disease (HD, IT15) gene. Huntington’s disease is a neurodegenerative disorder caused by a mutation on the HD gene, producing a polyglutamin (polyQ) expansion on the N-terminus of Htt. The mutant Htt leads to cytotoxicity in the striatal neuron (1). This cytotoxicity is modulated by proteolytic cleavage with caspases and calpains, producing N-terminal polyQ fragments. A phosphorylation of Htt may also regulate the cleavage and cytotoxicity of mutant Htt (2). The wild-type Htt up-regulates transcription of brain-derived neurotrophic factor (BDNF), a pro-survival factor for striatal neurons. This suggests restoring wild-type Htt level and increasing BDNF expression level can be therapeutic treatment for Huntington’s disease (3). Huntingtin is also cleaved by apopain, a cysteine protease product of death-gene for human, suggesting HD might be a disorder of inappropriate apoptosis (4). | | | Catalog # | H7965A | | Applications | Suitable for use in Flow Cytometry, Western Blot, Immunohistochemistry, Immunocytochemistry. Other applications not tested. | | Recommended Dilution | Flow Cytometry: 1:30 | | Western Blot: 1:10,000 | | Immunohistochemistry: 1:250 – 500 | | Immunocytochemistry: 1:50 – 100 | | Optimal dilutions to be determined by the researcher. | | Storage and Stability | May be stored at 4°C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot Freeze at -20°C. Aliquots are stable for at least 12 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer. | | CAS Number | n/a | | Clone Type | Monoclonal | | Isotype | IgG | | Clone No | 6k216 | | Host | Rabbit | | Source | Human | | Form | Supplied as a liquid. | | Purity | Supernatant | | Immunogen | A synthetic peptide corresponding to residues specific to the apopain cleavage site of human huntingtin protein | | Specificity | Recognizes human huntingtin protein | | | Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological. |
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