内质网分子伴侣SIL1抗体
规格：1mg/1ml
英文名： SIL1
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
分子量： 45kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human SIL1
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Guinea Pig, Cat,
细胞定位：
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: 内质网分子伴侣SIL1抗体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. 内质网分子伴侣SIL1抗体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.
研究领域：细胞生物 神经生物学 信号转导 细胞凋亡
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid