For research use only.
| Verified Samples |
Verified Samples in IHC: Human tonsil, Human esophagus cancer |
| Dilution | IHC 1:40-1:200 |
| Clonality | Polyclonal |
| Immunogen | Synthetic peptide of human HTT |
| Abbre | HTT |
| Synonyms | AI256365, C430023I11Rik, HD, HD protein, HDH, HTT, HUNTINGTON CHOREA, Huntingtin, Huntington disease protein, Huntington's disease protein homolog, IT 15, IT15, OTTMUSP00000026909, ZHD |
| Swissprot | |
| Cellular Localization | Cytoplasm. Nucleus. The mutant Huntingtin protein colocalizes with AKAP8L in the nuclear matrix of Huntington's disease neurons. |
| Concentration | 0.84 mg/mL |
| Buffer | PBS with 0.05% NaN3 and 40% Glycerol,pH7.4 |
| Purification Method | Antigen affinity purification |
| Research Areas | Neuroscience |
| Conjugation | Unconjugated |
| Storage | Store at -20°C Valid for 12 months. Avoid freeze / thaw cycles. |
| Shipping | The product is shipped with ice pack,upon receipt,store it immediately at the temperature recommended. |
| 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. |
Other Clones
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