Anti-Sacsin/FITC Conjugated抗体上海钰博生物科技有限公司

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产品名称: Anti-Sacsin/FITC Conjugated抗体
英文名称: Anti-Sacsin/FITC
抗体货号: YB--19363R-FITC
产品规格: 20ul/50ul/100ul/200ul
级    别: , 分析纯, 见说明书
产品产地: 中国/美国
品牌商标: Ybscience
价    格: 2980元
抗原: KLH conjugated synthetic peptide derived from human Sacsin
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: Human, Mouse, Rat, Pig, Rabbit,
见说明书conjugate:见说明书
Isotype:见说明书
应用范围: 科研使用
更新时间: 2019/7/12 10:25:00
详细资料:  实验方法技术资料
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诚信指数:2562点
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使用范围:仅限科研使用,不能应用于临床
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产品详细描述

 Anti-Sacsin/FITC Conjugated抗体

产品编号 YB-19363R-FITC
英文名称 Anti-Sacsin/FITC
中文名称 FITC标记的charlevoix-saguenay型痉挛性共济失调Sacsin蛋白抗体
别    名 DnaJ homolog subfamily C member 29; DNAJC29; SACS; SACS_HUMAN; Sacsin; spastic ataxia of Charlevoix-Saguenay.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  发育生物学  神经生物学  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Pig, Rabbit, 
产品应用 ICC=1:50-200 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 521kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Sacsin
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
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]

Function:
Phosphoinositide phosphatase that hydrolyzes PtdIns3P and PtdIns4P. Has low activity towards PtdIns(3,5)P2 (By similarity).
Co-chaperone which acts as a regulator of the Hsp70 chaperone machinery and may be involved in the processing of other ataxia-linked proteins.

Subcellular Location:
Cytoplasm. Predominantly cytoplasmic, a small portion is present in the nucleus and also shows a partial mitochondrial overlap with the mitochondrial marker Hsp60.

Tissue Specificity:
Highly expressed in the central nervous system. Also found in skeletal muscle and at low levels in pancreas.

DISEASE:
Defects in SACS are the cause of spastic ataxia Charlevoix-Saguenay type (SACS) [MIM:270550]. It is a neurodegenerative disease characterized by early-onset cerebellar ataxia, spasticity, retinal hypermyelination, pyramidal signs, and both axonal and demyelinating neuropathy with loss of sensory nerve conduction and reduced motor conduction velocities. Other features include dysarthria, distal muscle wasting, nystagmus, defect in conjugate pursuit ocular movements, retinal striation (from prominent retinal nerves) obscuring the retinal blood vessels in places, and the frequent presence of mitral valve prolapse.

Similarity:
Contains 1 HEPN domain.
Contains 1 J domain.
Contains 1 ubiquitin-like domain.

Database links:

Entrez Gene: 26278 Human

Entrez Gene: 50720 Mouse

Entrez Gene: 305940 Rat

Omim: 604490 Human

SwissProt: Q9NZJ4 Human

SwissProt: Q9JLC8 Mouse

Unigene: 159492 Human

Unigene: 440703 Mouse

Unigene: 446687 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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