SIL1 Antibody (PACO41318)
- SKU:
- PACO41318
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- ELISA
- Applications:
- WB
- Antibody Type:
- Polyclonal Antibody
- Conjugation:
- Unconjugated
Description
| Antibody Name: | SIL1 Antibody (PACO41318) |
| Antibody SKU: | PACO41318 |
| Size: | 50ug |
| Host Species: | Rabbit |
| Tested Applications: | ELISA, WB |
| Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:200-1:1000 |
| Species Reactivity: | Human, Mouse |
| Immunogen: | Recombinant Human Nucleotide exchange factor SIL1 protein (32-174AA) |
| Form: | Liquid |
| Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
| Purification Method: | >95%, Protein G purified |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Non-conjugated |
 | Western blot. All lanes: SIL1 antibody at 2µg/ml + Mouse kidney tissue. Secondary. Goat polyclonal to rabbit IgG at 1/10000 dilution. Predicted band size: 52 kDa. Observed band size: 52 kDa. |
| Background: | Required for protein translocation and folding in the endoplasmic reticulum (ER). Functions as a nucleotide exchange factor for the ER lumenal chaperone HSPA5. |
| Synonyms: | Nucleotide exchange factor SIL1 (BiP-associated protein) (BAP), SIL1 |
| UniProt Protein Function: | SIL1: Required for protein translocation and folding in the endoplasmic reticulum (ER). Functions as a nucleotide exchange factor for the ER lumenal chaperone HSPA5. Defects in SIL1 are a cause of Marinesco-Sjoegren syndrome (MSS). 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. Belongs to the SIL1 family.Protein type: Secreted, signal peptide; SecretedChromosomal Location of Human Ortholog: 5q31Cellular Component: endoplasmic reticulum; extracellular spaceMolecular Function: protein bindingDisease: Marinesco-sjogren Syndrome |
| UniProt Protein Details: | |
| NCBI Summary: | This gene encodes a resident endoplasmic reticulum (ER), N-linked glycoprotein with an N-terminal ER targeting sequence, 2 putative N-glycosylation sites, and a C-terminal ER retention signal. This protein functions as a nucleotide exchange factor for another unfolded protein response protein. Mutations in this gene have been associated with Marinesco-Sjogren syndrome. Alternate transcriptional splice variants have been characterized. [provided by RefSeq, Jul 2008] |
| UniProt Code: | Q9H173 |
| NCBI GenInfo Identifier: | 74733533 |
| NCBI Gene ID: | 64374 |
| NCBI Accession: | Q9H173.1 |
| UniProt Secondary Accession: | Q9H173,Q8N2L3, D3DQC2 |
| UniProt Related Accession: | Q9H173 |
| Molecular Weight: | 52,085 Da |
| NCBI Full Name: | Nucleotide exchange factor SIL1 |
| NCBI Synonym Full Names: | SIL1 nucleotide exchange factor |
| NCBI Official Symbol: | SIL1 |
| NCBI Official Synonym Symbols: | BAP; MSS; ULG5 |
| NCBI Protein Information: | nucleotide exchange factor SIL1 |
| UniProt Protein Name: | Nucleotide exchange factor SIL1 |
| UniProt Synonym Protein Names: | BiP-associated protein; BAP |
| Protein Family: | Silaffin |
| UniProt Gene Name: | SIL1 |
| UniProt Entry Name: | SIL1_HUMAN |
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