产品应用
应用说明
*最佳稀释倍数与浓度应由研究人员确认
WB: 免疫印迹
ICC/IF: 细胞染色
IHC-P: 石蜡切片
IHC-Fr: 冰冻切片
预期分子量
产品属性
形式
Liquid
存储溶液
PBS
存放说明
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
浓度
1 mg/ml (Please refer to the vial label for the specific concentration.)
免疫原种属
Human
免疫原
Recombinant fragment of human Huntingtin.
纯化方式
Affinity purified by Protein A.
偶联
Unconjugated
注意事项
仅供实验室使用。不适用于人类或动物的任何临床,治疗或诊断用途。不适合动物或人类食用。
相关讯息
别名
huntingtin , HD , IT15 , LOMARS
细胞定位
Cytoplasm,Nucleus
功能与背景
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. [provided by RefSeq, Jul 2016]
数据库
研究领域
產品图片
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GTX638832 WB Image
Wild-type (WT) and HTT knockout (KO) 293T cell extracts (30 μg) were separated by 5% SDS-PAGE, and the membrane was blotted with Huntingtin antibody [HL2483] (GTX638832) diluted at 1:1000. The HRP-conjugated anti-rabbit IgG antibody (GTX213110-01) was used to detect the primary antibody.
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GTX638832 WB Image
HeLa whole cell and nuclear extracts (30 μg) were separated by 5% SDS-PAGE, and the membrane was blotted with Huntingtin antibody [HL2483] (GTX638832) diluted at 1:1000. The HRP-conjugated anti-rabbit IgG antibody (GTX213110-01) was used to detect the primary antibody.
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