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折扣/货期| 货号：GTX31046 | 规格：50μg | 目录价：￥5300 |
产品详情
* 以下信息仅供参考，详情请以原厂网站为准
产品名称:
Huntingtin antibody [HDC8A4]
别名:
huntingtin , HD , IT15 , LOMARS
反应种属:
Human, Mouse, Rabbit
宿主来源:
Mouse
实验应用:
IHC-Fr, IP, WB
靶标/特异性:
Clone HDC8A4 reacts with an epitope corresponding to the HDC region (2703 - 2911 amino acids) of the huntingtin protein. This antibody recognizes both denatured and native huntingtin in human brain.
同种型:
IgG1
免疫原:
Recombinant protein corresponding to amino acids 2703 - 2911 of huntingtin.
克隆性:
Monoclonal
克隆号:
HDC8A4
纯化方式:
Protein G purified
偶联:
Unconjugated
产品浓度:
1.0 mg/ml (Please refer to the vial label for the specific concentration.)
保存温度:
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.
运输温度:
4°C
产品形式:
Liquid
存储溶液:
PBS, 0.09% Sodium azide.
生产商:
GeneTex
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]
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