Purified Rabbit Polyclonal Antibody
| 货号 | 规格 | 价格 |
|---|---|---|
| P103386 | ||
| 20µL | ¥588.00 | |
| 50µL | ¥1080.00 | |
| 100µL | ¥1780.00 |
立即购买
| Product Name | Anti-Huntingtin Rabbit pAb |
|---|---|
| Description | Purified Rabbit Polyclonal Antibody |
Application
|
WB, IHC-P/IF (Tissue-P), IF (Cell)/ICC, ELISA |
| Dilution | WB 1:500~1:2,000; IHC-P 1:50~1:200; IF 1:50~1:200 |
| Reactivity | Human, Mouse, Rat |
| Host | Rabbit |
| Clonality | Polyclonal |
| Isotype | IgG |
| Label | Unconjugated |
| Immunogen | Recombinant fusion protein containing a sequence corresponding to amino acids 435-635 of human Huntingtin (NP_002102.4). |
| Format | Affinity purified polyclonal antibody supplied in PBS with 0.02% sodium azide and 50% glycerol, pH 7.3. |
| Synonyms | HD; IT15; LOMARS; Huntingtin. |
|---|---|
| Molecular weight | Calculated MW: 348 kDa; Observed MW: 347 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 pAb 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. |
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