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蛋白质/抗原/多肽 >> 重组蛋白
重组charlevoix-saguenay型痉挛性共济失调Sacsin蛋白
重组charlevoix-saguenay型痉挛性共济失调Sacsin蛋白图片
交货期: 1周
索取资料及报价
交货期:1周
产品别名:Recombinant SACS
Recombinant sacsin molecular chaperone protein
产品介绍
基因名:

SACS


产品别名:

ARSACS; DNAJC29; PPP1R138; SPAX6; SACS; sacsin molecular chaperone; sacsin molecular chaperone; sacsin; dnaJ homolog subfamily C member 29; protein phosphatase 1, regulatory subunit 138; spastic ataxia of Charlevoix-Saguenay (sacsin); charlevoix-saguenay型痉挛性共济失调Sacsin蛋白;


背景信息:
This gene encodes the sacsin protein, which includes a UbL domain at the N-terminus, a DnaJ domain, and a HEPN domain at the C-terminus. The gene is highly expressed in the central nervous system, also found in skin, skeletal muscles and at low levels in the pancreas. This gene includes a very large exon spanning more than 12.8 kb. Mutations in this gene result in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), a neurodegenerative disorder characterized by early-onset cerebellar ataxia with spasticity and peripheral neuropathy. The authors of a publication on the effects of siRNA-mediated sacsin knockdown concluded that sacsin protects against mutant ataxin-1 and suggest that "the large multi-domain sacsin protein is able to recruit Hsp70 chaperone action and has the potential to regulate the effects of other ataxia proteins" (Parfitt et al., PubMed: 19208651). A pseudogene associated with this gene is located on chromosome 11. Alternative splicing of this gene results in multiple transcript variants. [provided by RefSeq, May 2013]

标签:His-tag   
分类:Recombinant  
类型:Protein   
偶联物:Unconjugated
内毒素水平:按批次,参阅瓶身标签
性状:Liquid
浓度:Batch dependent (Please refer to the vial label for the specific concentration.)
纯化类型:purified
内含物:不含防腐剂
应用:Positive Control;Immunogen;SDS-PAGE;WB.
储存:2-8°C不超过一个月,-80°C不超过12个月。避免反复冻融。
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。

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