Product Name	SIL1
Chinese Name	内质网分子伴侣SIL1抗体
Alias	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.
Research Area	Cell biology  Neurobiology  Signal transduction  Apoptosis
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Mouse,  (predicted: Human, Rat, Dog, Pig, Horse, )
Applications	WB=1:500-2000 ELISA=1:5000-10000  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	49kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human SIL1: 101-200/461
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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
Product Picture	Sample: Liver (Mouse) Lysate at 40 ug Primary: Anti-SIL1 (SL17493R) 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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