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Affinity Purified Rabbit Polyclonal Antibody (Pab)

Immunohistochemical analysis of paraffin-embedded human skeletal muscle tissue using AP8937C performed on the Leica® BOND RXm. Samples were incubated with primary antibody(1/500) for 1 hours at room temperature. A undiluted biotinylated CRF Anti-Polyvalent HRP Polymer antibody was used as the secondary antibody.
Overlay histogram showing Hela cells stained with AP8937C (green line). The cells were fixed with 2% paraformaldehyde (10 min) and then permeabilized with 90% methanol for 10 min. The cells were then icubated in 2% bovine serum albumin to block non-specific protein-protein interactions followed by the antibody (AP8937C , 1:25 dilution) for 60 min at 37ºC. The secondary antibody used was Goat-Anti-Rabbit IgG, DyLight® 488 Conjugated Highly Cross-Adsorbed(1583138) at 1/200 dilution for 40 min at 37ºC. Isotype control antibody (blue line) was rabbit IgG1 (1μg/1x10^6 cells) used under the same conditions. Acquisition of >10, 000 events was performed.
All lanes : Anti-GC Antibody (Center) at 1:2000 dilution Lane 1: 293T/17 whole cell lysate Lane 2: Hela whole cell lysate Lane 3: LNCaP whole cell lysate Lane 4: MCF-7 whole cell lysate Lane 5: U-2OS whole cell lysate Lysates/proteins at 20 µg per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 60 kDa Blocking/Dilution buffer: 5% NFDM/TBST.

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Product Information
Application Applications Legend: E=ELISA WB=Western Blotting IHC=Immunohistochemistry IHC-P=Immunohistochemistry (Paraffin) IP=Immunoprecipitation IF=Immunofluorescence IC=Immunochemistry ICC=Immunocytochemistry FC=Flow Cytometry DB=Dot Blot	WB, FC, IHC-P-Leica, E
Primary Accession	P04062
Other Accession	Q70KH2, Q2KHZ8
Reactivity	Human, Mouse
Predicted	Pig, Bovine, Rat, Canine, Rabbit
Host	Rabbit
Clonality	Polyclonal
Isotype	Rabbit IgG
Calculated MW	59716 Da
Antigen Region	337-365 aa

Additional Information
Gene ID	2629
Other Names	Glucosylceramidase, Acid beta-glucosidase, Alglucerase, Beta-glucocerebrosidase, Beta-GC, D-glucosyl-N-acylsphingosine glucohydrolase, Imiglucerase, GBA, GC, GLUC
Target/Specificity	This GC antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 337-365 amino acids of human GC.
Dilution	WB~~1:2000 FC~~1:25 IHC-P-Leica~~1:500 E~~Use at an assay dependent concentration.
Format	Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
Storage	Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
Precautions	GC Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name	GBA1 (HGNC:4177)
Synonyms	GBA, GC, GLUC
Function	Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramides/GlcCers (such as beta- D-glucosyl-(1<->1')-N-acylsphing-4-enine) into free ceramides (such as N-acylsphing-4-enine) and glucose (PubMed:15916907, PubMed:24211208, PubMed:32144204, PubMed:39395789, PubMed:9201993). Plays a central role in the degradation of complex lipids and the turnover of cellular membranes (PubMed:27378698). Through the production of ceramides, participates in the PKC-activated salvage pathway of ceramide formation (PubMed:19279011). Catalyzes the glucosylation of cholesterol, through a transglucosylation reaction where glucose is transferred from GlcCer to cholesterol (PubMed:24211208, PubMed:26724485, PubMed:32144204). GlcCer containing mono-unsaturated fatty acids (such as beta-D- glucosyl-N-(9Z-octadecenoyl)-sphing-4-enine) are preferred as glucose donors for cholesterol glucosylation when compared with GlcCer containing same chain length of saturated fatty acids (such as beta-D- glucosyl-N-octadecanoyl-sphing-4-enine) (PubMed:24211208). Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl 3-beta-D-glucoside to ceramide (Probable) (PubMed:26724485). Can also hydrolyze cholesteryl 3-beta-D- glucoside producing glucose and cholesterol (PubMed:24211208, PubMed:26724485, PubMed:39395789). Catalyzes the hydrolysis of galactosylceramides/GalCers (such as beta-D-galactosyl-(1<->1')-N- acylsphing-4-enine), as well as the transfer of galactose between GalCers and cholesterol in vitro, but with lower activity than with GlcCers (PubMed:32144204). Contrary to GlcCer and GalCer, xylosylceramide/XylCer (such as beta-D-xyosyl-(1<->1')-N-acylsphing-4- enine) is not a good substrate for hydrolysis, however it is a good xylose donor for transxylosylation activity to form cholesteryl 3-beta- D-xyloside (PubMed:33361282). Can also metabolize plant glycosyl phytosterols such as glucosylstigmasterol (PubMed:39395789).
Cellular Location	Lysosome membrane; Peripheral membrane protein; Lumenal side. Note=Interaction with saposin-C promotes membrane association (PubMed:10781797). Targeting to lysosomes occurs through an alternative MPR-independent mechanism via SCARB2 (PubMed:18022370).