Anti-SIL1 monoclonal antibody (DCABH-732)

Mouse anti-Human SIL1 monoclonal antibody for WB, IHC-P, ICC/IF Datasheet

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Specifications


Host Species
Mouse
Antibody Isotype
IgG1
Clone
2D5
Species Reactivity
Human
Immunogen
Recombinant full length protein of Human SIL1 produced in HEK293T cells (NP_071909).
Conjugate
Unconjugated

Applications


Application Notes
WB: 1/2000; IHC-P: 1/50; ICC/IF: 1/100;
*Suggested working dilutions are given as a guide only. It is recommended that the user titrates the product for use in their own experiment using appropriate negative and positive controls.

Target


Alternative Names
SIL1; SIL1 homolog, endoplasmic reticulum chaperone (S. cerevisiae); Marinesco Sjogren syndrome , MSS; nucleotide exchange factor SIL1; BAP; ULG5
Entrez Gene ID
UniProt ID

Product Background


Gene summary
SIL1 (SIL1 Nucleotide Exchange Factor) is a Protein Coding gene. Diseases associated with SIL1 include marinesco-sjogren syndrome and sjogrens syndrome. Among its related pathways are Protein processing in endoplasmic reticulum. GO annotations related to this gene include binding and unfolded protein binding. 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.
Antigen Description
Required for protein translocation and folding in the endoplasmic reticulum (ER). Functions as a nucleotide exchange factor for the ER lumenal chaperone HSPA5. Marinesco-Sjoegren syndrome (MSS) [MIM:248800]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry. Nucleotide exchange factor SIL1 is a protein that in humans is encoded by the SIL1 gene. 0The function about SIL1 antigen include binding; unfolded protein binding.
Pathway
Protein processing in endoplasmic reticulum, organism-specific biosystem; Protein processing in endoplasmic reticulum, conserved biosystem.

Citations


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References


Ichhaporia, VP; Sanford, T; et al. Sil1, a nucleotide exchange factor for BiP, is not required for antibody assembly or secretion. MOLECULAR BIOLOGY OF THE CELL 26:420-429(2015).

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