Rabbit anti-Human, Mouse SIL1 Polyclonal Antibody | anti-SIL1 antibody

SIL1, ID (Nucleotide Exchange Factor SIL1, BiP-associated Protein, BAP, UNQ545/PRO836) (AP)

Cat. Num. AAA6499523

• Gene Names: SIL1; BAP; MSS; ULG5
• Reactivity: Human, Mouse
• Applications: Western Blot, ELISA
• Purity: Purified by Immunoaffinity chromatography.
• Synonyms: SIL1; Polyclonal Antibody; ID (Nucleotide Exchange Factor SIL1; BiP-associated Protein; BAP; UNQ545/PRO836) (AP); anti-SIL1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human SIL1. Species Crossreactivity: mouse.
• Purity/Purification: Purified by Immunoaffinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phosphatase (AP).

• Applicable Applications for anti-SIL1 antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB: 1:100-1:500; ELISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide selected from the center region of human SIL1 (KLH).
• Conjugate: AP
• Note: Preservative Free
• Preparation and Storage: May be stored at 4 degree C for short-term only. Do not freeze alkaline phosphatase conjugates which could result in a substantial loss of enzymatic activity. Light sensitive. Aliquots are stable for at least 6 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western blot analysis of SIL1 Antibody (Center) in mouse kidney tissue lysates (35ug/lane).SIL1 (arrow) was detected using the purified Pab.)

Immunohistochemistry (IHC)

(Formalin-fixed and paraffin-embedded human hepatocarcinoma reacted with SIL1 Antibody (Center), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.)

Flow Cytometry (FC/FACS)

(Flow cytometric analysis of HepG2 cells using SIL1 Antibody (Center) (bottom histogram) compared to a negative control cell (top histogram). FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Product Categories/Family for anti-SIL1 antibody

Antibodies; Transport Proteins

References

1. Clark, H.F., et.al., Genome Res. 13(10), 2265-2270 (2003).

NCBI and Uniprot Product Information

• NCBI GI #: 11968009
• NCBI GeneID: 64374
• NCBI Accession #: NP_071909.1
• 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; BiP-associated protein; SIL1 homolog, endoplasmic reticulum chaperone
• 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