Huntingtin 抗体
Quick Overview for Huntingtin 抗体 (ABIN6943763)
抗原
See all Huntingtin (HTT) 抗体适用
宿主
克隆类型
标记
应用范围
克隆位点
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交叉反应
- 人, 小鼠, 大鼠
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纯化方法
- Purified by Protein A.
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免疫原
- Recombinant protein within human Huntingtin aa 1-150
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亚型
- IgG
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应用备注
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WB 1:300-5000
FCM 1:20-100
IHC-P 1:200-400
IF(ICC) 1:50-200 -
限制
- 仅限研究用
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状态
- Liquid
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浓度
- 1 μg/μL
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缓冲液
- Aqueous buffered solution containing 1xTBS ( pH 7.4), 1 % BSA, 40 %Glycerol and 0.05 % Sodium Azide.
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储存液
- ProClin
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注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
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储存条件
- 4 °C,-20 °C
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储存方法
- Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
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有效期
- 12 months
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- Huntingtin (HTT)
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别名
- Huntingtin
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背景
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Synonyms: Huntingtin, Huntington disease protein, HD protein, HTT, IT15, LOMARS
Background: 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. Huntingtin may play a role in microtubule-mediated transport or vesicle function.
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基因ID
- 3064
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UniProt
- P42858
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途径
- PI3K-Akt Signaling, Hormone Transport, Transition Metal Ion Homeostasis, Tube Formation, Protein targeting to Nucleus, Dicarboxylic Acid Transport
抗原
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