中文名称 神经性舞蹈病蛋白重组兔单克隆抗体
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.
研究领域 细胞生物 神经生物学 Alzheimer's
抗体来源 Rabbit
克隆类型 Monoclonal
克 隆 号 1F10
交叉反应 (predicted: Human, Mouse, Rat, )
产品应用 WB=1:200-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:20-100 (石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 347kDa
细胞定位 细胞核 细胞浆
性 状 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 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMed PubMed
产品介绍 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 hig