SIL1, Monoclonal Antibody

Monoclonal SIL1; Anti-SIL1; Sil1 Homolog Endoplasmic Reticulum Chaperone; BAP; MSS; ulG5; SIL1; SIL 1; SIL-1

For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

IgG1

1G7

Mouse

SIL1 antibody was purified by affinity chromatography.

Supplied in PBS buffer, pH 7.3, containing 1% BSA, 50% glycerol and 0.02% sodium azide.

500 ug-1 mg/ml (lot specific)

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.

Small volumes of anti-SIL1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Mouse monoclonal SIL1 antibody

Protein Modification & Stress Response

Flow Cytometry (FC/FACS), Immunofluorescence (IF), Immunohistochemistry (IHC), Western Blot (WB)

FC: 1:100
IF: 1:100
IHC: 1:50
WB: 1:2000

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

Immunohistochemical analysis of SIL1 protein in paraffin embedded Human kidney tissue using SIL1 antibody

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

52,085 Da

SIL1 nucleotide exchange factor

nucleotide exchange factor SIL1

Nucleotide exchange factor SIL1

BAP  [Similar Products]

SIL1_HUMAN

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]

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; Secreted

Chromosomal Location of Human Ortholog: 5q31

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

Molecular Function: unfolded protein binding

Biological Process: intracellular protein transport; protein folding

Disease: Marinesco-sjogren Syndrome

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