Mouse SIL1 Monoclonal Antibody | anti-SIL1 antibody

SIL1 antibody

Cat. Num. AAA834634

• Gene Names: SIL1; BAP; MSS; ULG5
• Applications: Immunofluorescence, Immunohistochemistry, Western Blot
• Purity: SIL1 antibody was purified by affinity chromatography.
• Synonyms: SIL1; Monoclonal Antibody; SIL1 antibody; Monoclonal SIL1; Anti-SIL1; Sil1 Homolog Endoplasmic Reticulum Chaperone; BAP; MSS; ulG5; SIL 1; SIL-1; anti-SIL1 antibody

• Host: Mouse
• Clonality: Monoclonal
• Isotype: IgG1
• Clone Number: 1C4
• Purity/Purification: SIL1 antibody was purified by affinity chromatography.
• Form/Format: Supplied in PBS buffer, pH 7.3, containing 1% BSA, 50% glycerol and 0.02% sodium azide.
• Concentration: 500 ug-1 mg/ml (varies by lot)
• Sequence Length: 461

• Applicable Applications for anti-SIL1 antibody: Immunofluorescence (IF), Immunohistochemistry (IHC), Western Blot (WB)
• Application Notes: IF: 1:100; IHC: 1:50; WB: 1:2000
• Biological Significance: SIL1 is 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 its gene have been associated with Marinesco-Sjogren syndrome.
• Cross-Reactivity: Human
• Immunogen: SIL1 antibody was raised in mouse using a full length recombinant protein of human SIL1 (NP_071909) produced in HEK293T cells, as the immunogen.
• Preparation and Storage: Store at 4 degree C for short term storage. Aliquot and store at -20 degree C for long term storage. Avoid repeated freeze/thaw cycles.

Immunofluorescence (IF)

(Immunofluorescent staining of COS7 cells transiently transfected with recombinant SIL1 protein using SIL1 antibody)

Western Blot (WB)

(Western Blot analysis of HEK293T cell lysates (5 ug) transfected with either recombinant SIL1 protein (Right) or empty vector (Left) detected with SIL1 antibody)

Immunohistochemistry (IHC)

(Immunohistochemical analysis of SIL1 protein in paraffin embedded Human liver tissue using SIL1 antibody)

Related Product Information for anti-SIL1 antibody

Mouse monoclonal SIL1 antibody

Product Categories/Family for anti-SIL1 antibody

Protein Modification & Stress Response

NCBI and Uniprot Product Information

• NCBI GI #: 11968009
• NCBI GeneID: 64374
• NCBI Accession #: NP_071909
• NCBI GenBank Nucleotide #: NM_022464.4
• UniProt Accession #: Q9H173; Q8N2L3; D3DQC2
• Molecular Weight: 52,085 Da
• NCBI Official Full Name: nucleotide exchange factor SIL1
• NCBI Official 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
• Protein Family: Silaffin
• UniProt Gene Name: SIL1
• UniProt Synonym Gene Names: BAP
• UniProt Entry Name: SIL1_HUMAN

NCBI Description

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 Description

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; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 5q31

Cellular Component: extracellular space; endoplasmic reticulum; endoplasmic reticulum lumen

Molecular Function: unfolded protein binding

Biological Process: intracellular protein transport; protein folding

Disease: Marinesco-sjogren Syndrome