内质网分子伴侣SIL1抗体

英文名称    SIL1    

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 reticulum chaperone (S. cerevisiae); SIL1 homolog endoplasmic reticulum chaperone; SIL1_HUMAN; UGL 5; ULG5.    

内质网分子伴侣SIL1抗体       

说 明 书    0.2ml      

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    Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Guinea Pig, Cat,     

产品应用    WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.    

分 子 量    45kDa    

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human SIL1    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4    

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.    

内质网分子伴侣SIL1抗体产品介绍    background:
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.

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

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

内质网分子伴侣SIL1抗体

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