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(xì)胞生物  神經(jīng)生物學(xué)  Alzheimer's   

抗體來源 Rabbit 

克隆類型 Monoclonal 

克 隆 號 1F10 

交叉反應(yīng) (predicted: Human, Mouse, Rat, ) 

產(chǎn)品應(yīng)用 WB=1:200-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:20-100 （石蠟切片需做抗原修復(fù)）

not yet tested in other applications.

optimal dilutions/concentrations should be determined by the end user. 

分 子 量 347kDa 

細(xì)胞定位 細(xì)胞核 細(xì)胞漿  

性    狀 Liquid 

濃    度 1mg/ml 

免 疫 原 KLH conjugated synthetic peptide derived from human Huntingtin:  

亞    型 IgG 

純化方法 affinity purified by Protein A 

儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. 

保存條件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. 

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 hig

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