Purified Recombinant Rabbit Monoclonal Antibody
| 货号 | 规格 | 价格 |
|---|---|---|
| R014030 | ||
| 20µL | ¥588.00 | |
| 50µL | ¥1080.00 | |
| 100µL | ¥1780.00 |
立即购买
![Anti-Huntingtin Rabbit mAb [48H75B12]](https://files.epizyme.cn:5005/uploads/productimage/R014030-1.png)
![Anti-Huntingtin Rabbit mAb [48H75B12]](https://files.epizyme.cn:5005/uploads/productimage/R014030-2.png)
| Product Name | Anti-Huntingtin Rabbit mAb |
|---|---|
| Description | Purified Recombinant Rabbit Monoclonal Antibody |
Application
|
WB, IHC-P/IF (Tissue-P), ELISA |
| Dilution | WB 1:1,000~1:2,000; IHC-P 1:200; IF 1:100~1:200 |
| Reactivity | Mouse, Rat |
| Host | Rabbit |
| Clonality | Monoclonal |
| Clone No. | 48H75B12 |
| Isotype | IgG |
| Label | Unconjugated |
| Immunogen | A synthesized peptide derived from human Huntingtin |
| Format | Affinity purified monoclonal antibody supplied in PBS with 0.01% sodium azide and 50% glycerol, pH 7.3. |
| Synonyms | HD, IT15, LOMARS, HD protein, HD_HUMAN, HDH, HTT, HUNTINGTON CHOREA, Huntington disease protein, Huntington's disease protein homolog, IT 15, IT15, OTTMUSP00000026909, ZHD, AI256365, C430023I11Rik. |
|---|---|
| Molecular weight | Calculated MW: 348 kDa; Observed MW: 348 kDa |
| Uniprot ID | P42858 |
| Gene ID | 3064 |
| Storage | Shipped on wet ice. Store at -20℃. Stable for 24 months from date of receipt. Aliquoting is unnecessary for -20℃ storage. |
| Precautions | Anti-Huntingtin Rabbit mAb [48H75B12] is for research use only and not for use in diagnostic or therapeutic procedures. |
| Background | Huntingtin is a disease gene linked to Huntington's disease, a neurodegenerative disorder characterized by loss of striatal neurons. This is thought to be caused by an expanded, unstable trinucleotide repeat in the huntingtin gene, which translates as a polyglutamine repeat in the protein product. A fairly broad range of trinucleotide repeats (9-35) has been identified in normal controls, and repeat numbers in excess of 40 have been described as pathological. The huntingtin locus is large, spanning 180 kb and consisting of 67 exons. The huntingtin gene is widely expressed and is required for normal development. It is expressed as 2 alternatively polyadenylated forms displaying different relative abundance in various fetal and adult tissues. The larger transcript is approximately 13.7 kb and is expressed predominantly in adult and fetal brain whereas the smaller transcript of approximately 10.3 kb is more widely expressed. The genetic defect leading to Huntington's disease may not necessarily eliminate transcription, but may confer a new property on the mRNA or alter the function of the protein. One candidate is the huntingtin-associated protein-1, highly expressed in brain, which has increased affinity for huntingtin protein with expanded polyglutamine repeats. This gene contains an upstream open reading frame in the 5' UTR that inhibits expression of the huntingtin gene product through translational repression. [provided by RefSeq, Jul 2016] |
|---|
| Cellular Location | Cytoplasm. Nucleus. The mutant Huntingtin protein colocalizes with AKAP8L in the nuclear matrix of Huntington's disease neurons. |
|---|
| Tissue Location | Expressed 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. |
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