| 產(chǎn)品編號 | bsm-54305R |
| 英文名稱(chēng) | Huntingtin Recombinant Rabbit mAb |
| 中文名稱(chēng) | 神經(jīng)性舞蹈病蛋白重組兔單抗 |
| 別 名 | HD; HD protein; HD_HUMAN; HDH; HTT; Huntingtin; HUNTINGTON CHOREA; Huntington disease protein; Huntington's disease protein homolog; IT 15; IT15; OTTMUSP00000026909; ZHD; AI256365; C430023I11Rik. |
| 研究領(lǐng)域 | 細胞生物 神經(jīng)生物學(xué) Alzheimer's |
| 抗體來(lái)源 | Rabbit |
| 克隆類(lèi)型 | Recombinant |
| 克 隆 號 | 1F10 |
| 交叉反應 | Human,Mouse,Rat |
| 產(chǎn)品應用 | WB=1:500-2000,IHC-P=1:50-200,IHC-F=1:50-200,IF=1:50-200,ICC/IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 347 kDa |
| 檢測分子量 | |
| 細胞定位 | 細胞核 細胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | A synthesized peptide derived from human Huntingtin: 1-47 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
Huntingtin is a protein that contains a polyglutamine region. When the number of glutamine repeats exceeds 35, the gene encodes a version of Huntingtin that leads to Huntington’s disease (HD). When the polyglutamine stretch is mutated, Huntingtin acts within the nucleus to induce neurodegeneration by a cell-specific apoptotic mechanism. Loss of Huntingtin activity is unlikely to be the cause of HD, and it has been proposed that the expanded glutamine repeat region may induce an abnormal interaction between the mutant protein and other cellular proteins. Huntingtin interacts with a variety of proteins including HAP1, glyceraldehyde phosphate dehydrogenase (GAPDH) and HIP1. Function: May play a role in microtubule-mediated transport or vesicle function. Subunit: Binds SH3GLB1 (By similarity). Interacts through its N-terminus with PRPF40A. Interacts with PQBP1, SETD2 and SYVN. Interacts with PFN1. Subcellular Location: Cytoplasm. Nucleus. The mutant Huntingtin protein colocalizes with AKAP8L in the nuclear matrix of Huntington's disease neurons. Tissue Specificity: 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. Post-translational modifications: Cleaved by apopain downstream of the polyglutamine stretch. The resulting N-terminal fragment is cytotoxic and provokes apoptosis. Forms with expanded polyglutamine expansion are specifically ubiquitinated by SYVN1, which promotes their proteasomal degradation. DISEASE: Defects in HTT are the cause of Huntington disease (HD) [MIM:143100]. HD is an autosomal dominant neurodegenerative disorder characterized by involuntary movements (chorea), general motor impairment, psychiatric disorders and dementia. Onset of the disease occurs usually in the third or fourth decade of life and symptoms progressively worsen leading to death in 10 to 20 years. Onset and clinical course depend on the degree of poly-Gln repeat expansion, longer expansions resulting in earlier onset and more severe clinical manifestations. HD affects 1 in 10,000 individuals of European origin. Neuropathology of Huntington disease displays a distinctive pattern with loss of neurons, especially in the caudate and putamen (striatum). Similarity: Belongs to the huntingtin family. Contains 10 HEAT repeats. SWISS: P42858 Gene ID: 3064 Database links: Entrez Gene: 3064 Human Entrez Gene: 15194 Mouse Omim: 143100 Human Omim: 613004 Human SwissProt: P42858 Human SwissProt: P42859 Mouse Unigene: 518450 Human Unigene: 209071 Mouse Unigene: 482929 Mouse Unigene: 11193 Rat |
| 產(chǎn)品圖片 |
25 ug total protein per lane of various lysates (see on figure) probed with Huntingtin monoclonal antibody, unconjugated (bsm-54305R) at 1:2000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.
Paraformaldehyde-fixed, paraffin embedded Human Cerebellum; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Huntingtin Monoclonal Antibody, Unconjugated(bsm-54305R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Rat Cerebellum; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Huntingtin Monoclonal Antibody, Unconjugated(bsm-54305R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Human Testicles; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Huntingtin Monoclonal Antibody, Unconjugated(bsm-54305R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Rat Testicles; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Huntingtin Monoclonal Antibody, Unconjugated(bsm-54305R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Human Cerebrum; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Huntingtin Monoclonal Antibody, Unconjugated(bsm-54305R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Rat Cerebrum; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Huntingtin Monoclonal Antibody, Unconjugated(bsm-54305R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Mouse Cerebrum; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Huntingtin Monoclonal Antibody, Unconjugated(bsm-54305R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
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