Goat Polyclonal to Human BAP / SIL1 | MyBioSource MBS242612 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Goat Polyclonal to Human BAP / SIL1

catalog :

MBS242612

quantity :

0.05 mg

price :

495 USD

clonality :

polyclonal

host :

goat

conjugate :

nonconjugated

reactivity :

human, dog

application :

western blot, ELISA, immunohistochemistry, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS242612

products type :

Antibody

products full name :

Goat Polyclonal to Human BAP / SIL1

products short name :

BAP / SIL1

products name syn :

Anti-BAP / SIL1 Antibody (C-Terminus) IHC-plus; SIL1; BiP-associated protein; BAP; Marinesco-Sjogren syndrome; MSS; ULG5; Human BAP; SIL1

other names :

nucleotide exchange factor SIL1; Nucleotide exchange factor SIL1; nucleotide exchange factor SIL1; BiP-associated protein; SIL1 homolog, endoplasmic reticulum chaperone; SIL1 nucleotide exchange factor; BiP-associated protein; BAP

products gene name :

BAP

products gene name syn :

SIL1

other gene names :

SIL1; SIL1; BAP; MSS; ULG5; BAP

uniprot entry name :

SIL1_HUMAN

clonality :

Polyclonal

host :

Goat

reactivity :

Gorilla, Human. Predicted Reactivity: Monkey, Bat, Dog, Horse (at least 90% immunogen sequence identity)

sequence length :

461

specificity :

Human SIL1. Variants (NP_071909.1; NP_001032722.1) encode the same isoform.

purity :

Immunoaffinity Purified

form :

Tris-buffered saline, pH 7.3, 0.5% BSA, 0.02% sodium azide

concentration :

0.5 mg/ml

storage stability :

Store at -20 degree C. Minimize freezing and thawing.

tested application :

Immunohistochemistry (IHC - Paraffin), Western Blot (WB), ELISA (EIA)

app notes :

ELISA (1:8000), IHC-P (5 ug/ml), WB (1 - 2 ug/ml). Usage: Immunohistochemistry: was validated for use in immunohistochemistry on a panel of 21 formalin-fixed, paraffin-embedded (FFPE) human tissues after heat induced antigen retrieval in pH 6.0 citrate buffer. After incubation with the primary anti.

other info1 :

Target Species: Human. Immunogen Description: Synthetic peptide C-ELLGSVNSLLKELR from the C-terminus of human SIL1 (NP_071909.1; NP_001032722.1). Percent identity by BLAST analysis: Human, Gorilla, Monkey (100%); Marmoset, Dog, Bat, Horse (93%); Hamster, Panda, Rabbit (86%).

other info2 :

Immunogen Type: Synthetic peptide. Immunogen: BAP / SIL1 antibody was raised against synthetic peptide C-ELLGSVNSLLKELR from the C-terminus of human SIL1 (NP_071909.1; NP_001032722.1). Percent identity by BLAST analysis: Human, Gorilla, Monkey (100%); Marmoset, Dog, Bat, Horse (93%); Hamster, Panda, Rabbit (86%). Antigen Modification: C-Terminus

ncbi gi num :

11968009

ncbi acc num :

NP_071909.1

ncbi gb acc num :

NM_022464.4

uniprot acc num :

Q9H173

ncbi mol weight :

52,085 Da

ncbi pathways :

Protein Processing In Endoplasmic Reticulum Pathway (167325); Protein Processing In Endoplasmic Reticulum Pathway (167190)

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 summary :

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

size1 :

0.05 mg

price1 :

495 USD

more info or order :

MyBioSource product webpage