catalog number :
MBS820370
products full name :
Anti-Beta-glucosidase 2 Antibody
products short name :
[Beta-glucosidase 2]
products name syn :
[G19P1; Glucosidase 2 subunit beta; 80K-H protein; Glucosidase II subunit beta; Protein kinase C substrate 60.1 kDa protein heavy chain; PKCSH]
other names :
[glucosidase 2 subunit beta isoform 2; Glucosidase 2 subunit beta; glucosidase 2 subunit beta; hepatocystin; AGE-binding receptor 2; glucosidase II subunit beta; protein kinase C substrate, 80 Kda protein; protein kinase C substrate 60.1 kDa protein heavy chain; protein kinase C substrate 80K-H; 80K-H protein; Glucosidase II subunit beta; Protein kinase C substrate 60.1 kDa protein heavy chain; PKCSH]
products gene name :
[PRKCSH]
other gene names :
[PRKCSH; PRKCSH; PCLD; PLD1; G19P1; PKCSH; AGE-R2; G19P1; PKCSH]
uniprot entry name :
GLU2B_HUMAN
specificity :
Recognizes endogenous levels of Beta-glucosidase 2 protein.
purity :
The antibody was purified by immunogen affinity chromatography.
form :
Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.
storage stability :
Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles.
tested application :
Western Blot (WB), Immunofluorescence (IF), Immunocytochemsitry (ICC)
app notes :
WB (1/500 - 1/1000); IF/ICC (1/100 - 1/500)
image1 heading :
Western Blot (WB)
image2 heading :
Immunofluorescence (IF)
other info2 :
Source: Rabbit. Immunogen: KLH-conjugated synthetic peptide encompassing a sequence within the center region of human Beta-glucosidase 2. The exact sequence is proprietary.
products description :
Rabbit polyclonal antibody to Beta-glucosidase 2
ncbi acc num :
NP_001001329.1
ncbi gb acc num :
NM_001001329.2
ncbi mol weight :
59,178 Da
ncbi pathways :
Advanced Glycosylation Endproduct Receptor Signaling Pathway (187092); Asparagine N-linked Glycosylation Pathway (161013); Calnexin/calreticulin Cycle Pathway (187218); Immune System Pathway (106386); Innate Immune System Pathway (106387); Metabolism Of Proteins Pathway (106230); N-glycan Trimming In The ER And Calnexin/Calreticulin Cycle Pathway (187217); Post-translational Protein Modification Pathway (161012); Protein Processing In Endoplasmic Reticulum Pathway (167325); Protein Processing In Endoplasmic Reticulum Pathway (167190)
ncbi summary :
This gene encodes the beta-subunit of glucosidase II, an N-linked glycan-processing enzyme in the endoplasmic reticulum. The encoded protein is an acidic phosphoprotein known to be a substrate for protein kinase C. Mutations in this gene have been associated with the autosomal dominant polycystic liver disease. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014]
uniprot summary :
PRKCSH: Regulatory subunit of glucosidase II. Defects in PRKCSH are a cause of polycystic liver disease (PCLD). PCLD is an autosomal dominant disorder and is characterized by the presence of multiple liver cysts of biliary epithelial origin. PCLD is a distinct clinical and genetic entity that can occur independently from autosomal dominant polycystic kidney disease (ADPKD), which in a considerable but uncertain proportion of cases is associated with hepatic cysts. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Adaptor/scaffold. Chromosomal Location of Human Ortholog: 19p13.2. Cellular Component: endoplasmic reticulum lumen; endoplasmic reticulum; intracellular. Molecular Function: protein kinase C binding; phosphoprotein binding; calcium ion binding. Biological Process: cellular protein metabolic process; protein folding; innate immune response; protein amino acid N-linked glycosylation via asparagine; N-glycan processing; post-translational protein modification. Disease: Polycystic Liver Disease