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Anti -Huntingtin Protein (Huntington Disease)
Anti -Huntingtin Protein (Huntington Disease)
Pricing
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| Clone |
Host |
Grade |
Applications |
| Monoclonal |
Mouse |
Affinity Purified |
B Gel |
|
| 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 in the number of trinucleotide repeats 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. | | | Catalog # | H7965-01F | | Applications | Suitable for use in Dot Blot and Western Blot. Other applications not tested. | | Recommended Dilution | 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, add sterile glycerol (40-50%), aliquot and store 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 | IgG2b | | Clone No | 8D100 | | Host | Mouse | | Source | Human | | Concentration | ~0.1mg/ml | | Form | Supplied as a liquid in PBS, 7.4, 1% BSA and 0.05% sodium azide. | | Purity | Purified using Protein G column chromatography from culture supernatant of hybridoma cultured in a medium containing bovine IgG-depleted (~95%) fetal bovine serum. | | Immunogen | Partial sequence of recombinant full-length protein to human HD | | Specificity | Recognizes human HD. | | | 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. | | Alternate names | HD, HTT, IT15 |
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