FITC标记的神经性舞蹈病蛋白抗体
产品名称： FITC标记的神经性舞蹈病蛋白抗体
英文名称： Anti-Huntingtin/FITC
产品编号： HZ-11699R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-Huntingtin/FITC Conjugated antibody
FITC标记的神经性舞蹈病蛋白抗体
| 产品编号 | bs-11699R-FITC |
| 英文名称 | Anti-Huntingtin/FITC |
| 中文名称 | FITC标记的神经性舞蹈病蛋白抗体 |
| 别 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. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 细胞生物 神经生物学 Alzheimer's |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 347kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Huntingtin |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 产品介绍 | background: 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. Database links: Entrez Gene: 3064 Human Entrez Gene: 15194 Mouse Entrez Gene: 29424 Rat Omim: 143100 Human Omim: 613004 Human SwissProt: P42858 Human SwissProt: P42859 Mouse SwissProt: P51111 Rat Unigene: 518450 Human Unigene: 209071 Mouse Unigene: 482929 Mouse Unigene: 11193 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
亨廷顿蛋白是一种含有多聚谷氨酰胺区的蛋白质。当谷氨酰胺重复的数目超过35时，该基因编码亨廷顿的一个版本，导致亨廷顿氏病（HD）。当多聚谷氨酰胺伸展发生突变时，亨廷顿作用于细胞核内，通过细胞特异性凋亡机制诱导神经退行性变。HuntTin活性的丧失不太可能是HD的原因，并且已经提出谷氨酰胺重复区可能诱导突变蛋白与其他细胞蛋白之间的异常相互作用。亨廷顿蛋白与多种蛋白质相互作用，包括HAP1、甘油醛磷酸脱氢酶（GAPDH）和HIP1。