Anti-Huntingtin antibody produced in rabbit,100UL,SAB5701184-100UL,Sigma

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订货号 0BM3554
品牌型号 Sigma SAB5701184-100UL
货期 询货期
最小订货量 1件
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产品介绍 Product Description

General description

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]


Immunogen

A synthesized peptide derived from human Huntingtin


Physical form

Buffer: PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.


技术参数 Specifications
biological sourcerabbit
Quality Level100
antibody product typeprimary antibodies
clonemonoclonal
formliquid
species reactivityhuman, mouse
concentration0.63 mg/mL
technique(s)immunohistochemistry: 1:50-1:200,western blot: 1:500-1:2000
UniProt accession no.P42858
shipped inwet ice
storage temp.−20°C
target post-translational modificationunmodified
Gene Informationhuman ... HTT(3064)
长度(mm)
宽度(mm)
高度(mm)
重量(kg)
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