Key features and details
- Goat polyclonal to SIL1
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Preservative: 0.02% Sodium azide
Constituents: 0.5% Tris buffered saline, 0.5% BSA
Concentration information loading...
纯度Immunogen affinity purified
纯化说明Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Primary antibody说明SIL1 is a resident endoplasmic reticulum (ER) glycoprotein that interacts with the ATPase domain of BIP and enhances nucleotide exchange.
Our Abpromise guarantee covers the use of ab5639 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use a concentration of 1 - 2 µg/ml. Detects a band of approximately 55-60 kDa (predicted molecular weight: 53 kDa).
A 1 hour primary incubation is recommended for this product.
功能Required for protein translocation and folding in the endoplasmic reticulum (ER). Functions as a nucleotide exchange factor for the ER lumenal chaperone HSPA5.
组织特异性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.
疾病相关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.
序列相似性Belongs to the SIL1 family.
发展阶段Expressed in fetal kidney, fetal lung, fetal liver and at low levels in fetal brain.
细胞定位Endoplasmic reticulum lumen.
- Information by UniProt
- BAP antibody
- BiP associated protein antibody
- BiP-associated protein antibody
ab5639 staining (1
µg/ml) of Human Kidney lysate (RIPA buffer, 30 µg total protein per lane). Primary incubated for 1 hour. Detected by western blot using chemiluminescence.
ab5639 staining (1µg/ml) of Human Kidney lysate (RIPA buffer, 30µg total protein per lane). Primary incubated for 1 hour. Detected by western blot using chemiluminescence.
ab5639 被引用在 6 文献中.
- Gatz C et al. Identification of Cellular Pathogenicity Markers for SIL1 Mutations Linked to Marinesco-Sjögren Syndrome. Front Neurol 10:562 (2019). PubMed: 31258504
- Xu H et al. SIL1 functions as an oncogene in glioma by AKT/mTOR signaling pathway. Onco Targets Ther 11:3775-3783 (2018). PubMed: 29997438
- Filézac de L'Etang A et al. Marinesco-Sjögren syndrome protein SIL1 regulates motor neuron subtype-selective ER stress in ALS. Nat Neurosci 18:227-38 (2015). IHC-FoFr, IHC-P ; Mouse, Human . PubMed: 25559081
- Anttonen AK et al. Novel SIL1 mutations and exclusion of functional candidate genes in Marinesco-Sjögren syndrome. Eur J Hum Genet 16:961-9 (2008). ICC/IF ; Mouse . PubMed: 18285827
- Liu Y et al. The orphan receptor tyrosine kinase Ror2 promotes osteoblast differentiation and enhances ex vivo bone formation. Mol Endocrinol 21:376-87 (2007). PubMed: 17095577
- Anttonen AK et al. The gene disrupted in Marinesco-Sjögren syndrome encodes SIL1, an HSPA5 cochaperone. Nat Genet 37:1309-11 (2005). IHC-Fr ; Human . PubMed: 16282978