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GAA Recombinant Rabbit Monoclonal Antibody

Catalog Number:OM670620
OM670620
Discount Price
OM670620-100ul
100ul
OM670620-50ul
50ul
OM670620-20ul
20ul
Product Profile
Product Name GAA Recombinant Rabbit Monoclonal Antibody
Antibody Type Primary Antibodies
Immunogen Recombinant protein within Human GAA aa 120-230 / 952.
Key Feature
Clonality Monoclonal
Isotype IgG
Host Species Rabbit
Tested Applications FCIHCWB
WB:1:500-1:2000
IF:1:50-1:200
IHC:1:50-1:1000
Species Reactivity Human
Concentration 1mg/ml
Purification Protein A
Target Information
Gene Symbol GAA
Gene Synonyms IOPD
LOPD
LYAG
Gene Full Name alpha glucosidase
Gene Summary This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2016]
Alternative Names 70 kDa lysosomal alpha-glucosidase antibody
Acid alpha glucosidase antibody
Acid maltase antibody
Aglucosidase alfa antibody
Alpha glucosidase antibody
GAA antibody
Glucosidase alpha acid (Pompe disease glycogen storage disease type II) antibody
Glucosidase alpha acid antibody
Glucosidase alpha antibody
LYAG antibody
70 kDa lysosomal alpha-glucosidase antibody
Acid alpha glucosidase antibody
Acid maltase antibody
Aglucosidase alfa antibody
Alpha glucosidase antibody
GAA antibody
Glucosidase alpha acid (Pompe disease glycogen storage disease type II) antibody
Glucosidase alpha acid antibody
Glucosidase alpha antibody
LYAG antibody
LYAG_HUMAN antibody
Lysosomal alpha glucosidase antibody
Molecular Weight(MW) 105/76/70 kDa
Cellular Localization Lysosome, lysosome membrane.
Application

WB

Western blot analysis of GAA on different lysates with Rabbit anti-GAA antibody at 1/2,000 dilution. Lane 1: HepG2 cell lysate, Lane 2: MCF7 cell lysate, Lysates/proteins at 20 µg/Lane. Exposure time: 6 seconds; 4-20% SDS-PAGE gel. Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody at 1/2,000 dilution was used in 5% NFDM/TBST at 4℃ overnight. Goat Anti-Rabbit IgG - HRP Secondary Antibody at 1/50,000 dilution was used for 1 hour at room temperature.

WB

Western blot analysis of GAA on different lysates with Rabbit anti-GAA antibody at 1/1,000 dilution. Lane 1: HCT 116-si NT cell lysate, Lane 2: HCT 116-si GAA cell lysate, Lysates/proteins at 10 µg/Lane. Exposure time: 10 seconds; ECL: K1801; 4-20% SDS-PAGE gel. Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody at 1/1,000 dilution was used in 5% NFDM/TBST at 4℃ overnight. Goat Anti-Rabbit IgG - HRP Secondary Antibody at 1/50,000 dilution was used for 1 hour at room temperature.

IHC

Immunohistochemical analysis of paraffin-embedded human liver carcinoma tissue with Rabbit anti-GAA antibody at 1/200 dilution. The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody at 1/200 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.

IHC

Immunohistochemical analysis of paraffin-embedded human liver tissue with Rabbit anti-GAA antibody at 1/1,000 dilution. The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody at 1/1,000 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.

IHC

Immunohistochemical analysis of paraffin-embedded human placenta tissue with Rabbit anti-GAA antibody at 1/1,000 dilution. The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody at 1/1,000 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.

FC

Flow cytometric analysis of GAA was done on HepG2 cells. The cells were fixed, permeabilized and stained with the primary antibody (red). After incubation of the primary antibody at room temperature for an hour, the cells were stained with a Alexa Fluor 488-conjugated Goat anti-Rabbit IgG Secondary antibody at 1/1000 dilution for 30 minutes.Unlabelled sample was used as a control (cells without incubation with primary antibody; black).
Application Notes WB:1:500-1:2000
IF:1:50-1:200
IHC:1:50-1:1000
Additional Information
Form Liquid
Storage Instructions Shipped at 4℃. Store at +4℃ short term (1-2 weeks). It is recommended to aliquot into single-use upon delivery. Store at -20℃ long term.
Storage Buffer PBS (pH7.4), 0.1% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
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