Huntingtin Rabbit mAb  (货号:B29534)

说明书

货号:B29534

规格价格
50ul ¥1080.00 加购物车
100ul ¥2050.00 加购物车
反应 Human,Mouse,Rat
宿主 Rabbit
克隆性 Monoclonal
应用 WBIHCIF/ICCFC
推荐浓度 WB: 1:500 - 1:2000
IHC: 1:50 - 1:200
IF/ICC: 1:50 - 1:200
FC: 1:20 - 1:50
理论分子量 347kDa
实测分子量 348kDa
形式 Liquid
保存条件 Store at -20℃. Avoid freeze / thaw cycles.
Buffer: PBS with 0.75% BSA,50% glycerol,pH7.3.
偶联物 Unconjugated
阳性对照 HeLa,SH-SY5Y,U-251MG,Mouse brain
细胞定位 Cytoplasm,Nucleus
纯化 Affinity purification

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抗原信息

抗原信息 Recombinant fusion protein.
序列 Email For Sequence

靶点信息

研究背景 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 of trinucleotide repeats (9-35) 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.
基因ID 3064
基因名 HTT
Swiss P42858
别名 HD;IT15;HTT;Huntingtin
组织表达 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.
功能 May play a role in microtubule-mediated transport or vesicle function.
研究领域

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