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RabbitAnti-SIL1 Rabbit pAb  antibody (bs-17493R)
~~~促销,代码KXJ230206~~~
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-17493R
英文名称 SIL1 Rabbit pAb
中文名称 内质网分子伴侣SIL1抗体
别    名 BAP; BiP associated protein; BiP-associated protein; Endoplasmic reticulum chaperone SIL 1; Endoplasmic reticulum chaperone SIL1; MSS; Nucleotide exchange factor SIL 1; Nucleotide exchange factor SIL1; SIL 1; sil1; SIL1 homolog; SIL1 homolog endoplasmic r  
研究领域 细胞生物  神经生物学  信号转导  细胞凋亡  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Mouse,  (predicted: Human, Rat, Dog, Pig, Horse, )
产品应用 WB=1:500-2000 ELISA=1:5000-10000 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 49 kDa
检测分子量
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SIL1: 101-200/461 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 SIKE interacts with IKK-epsilon (IKBKE; MIM 605048) and TBK1 (MIM 604834) and acts as a suppressor of TLR3 (MIM 603029) and virus-triggered interferon activation pathways (Huang et al., 2005 [PubMed 16281057]).[supplied by OMIM, Mar 2008]

Function:
Required for protein translocation and folding in the endoplasmic reticulum (ER). Functions as a nucleotide exchange factor for the ER lumenal chaperone HSPA5.

Subcellular Location:
Endoplasmic reticulum lumen.

Tissue Specificity:
Highly expressed in tissues which produce large amounts of secreted proteins such as kidney, liver and placenta. Also expressed in colon, heart, lung, ovary, pancreas, peripheral leukocyte, prostate, spleen and thymus. Expressed at low levels throughout the brain.

Post-translational modifications:
N-glycosylated.

DISEASE:
Defects in SIL1 are a cause of Marinesco-Sjoegren syndrome (MSS) [MIM:248800]. MSS is an autosomal recessive multisystem disorder which is characterized by cerebellar ataxia due to cerebellar atrophy, with Purkinje and granule cell loss and myopathy featuring marked muscle replacement with fat and connective tissue. Other cardinal features include bilateral cataracts, hypergonadotrophic hypogonadism and mild to severe mental retardation. Skeletal abnormalities, short stature, dysarthria, strabismus and nystagmus are also frequent findings. Mutational inactivation of this protein may result in ER stress-induced cell death signaling or malfunctioning chaperone machineries that mishandle client proteins which are critical for the organs targeted in MSS.

Similarity:
Belongs to the SIL1 family.

SWISS:
Q9H173

Gene ID:
64374

Database links:

Entrez Gene: 64374 Human

Entrez Gene: 81500 Mouse

Entrez Gene: 291673 Rat

Omim: 608005 Human

SwissProt: Q9H173 Human

SwissProt: Q9EPK6 Mouse

SwissProt: Q6P6S4 Rat

Unigene: 483521 Human

Unigene: 291482 Mouse

Unigene: 103851 Rat



产品图片
Sample: Liver (Mouse) Lysate at 40 ug Primary: Anti-SIL1 (bs-17493R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 49 kD Observed band size: 59 kD
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