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charlevoix-saguenay型痉挛性共济失调Sacsin蛋白抗体
charlevoix-saguenay型痉挛性共济失调Sacsin蛋白抗体图片
交货期: 1周
索取资料及报价
交货期: 1周
半岛bd体育手机客户端 别名: Anti-SACS
Anti-sacsin molecular chaperone Antibody
半岛bd体育手机客户端 介绍
靶标:

SACS


半岛bd体育手机客户端 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).

宿主:Rbt
类型:Pab
同种型:IgG
应用:WB
纯化方式:亲和纯化
偶联物:Unconjugated
性状:液体
存储溶液:参阅说明书
浓度:Batch dependent (Please refer to the vial label for the specific concentration.)
稀释比例: Optimal dilutions/concentrations should be determined by the end user
储存:经常使用则4°C保存。-20°C保存不超过两年。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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