Galactosidase alpha Mouse Monoclonal Antibody [A7C4]
Catalog# HA600088
Galactosidase alpha Mouse Monoclonal Antibody [A7C4]
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WB
-
IHC-P
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Human
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unconjugated
Overview
Product Name
Galactosidase alpha Mouse Monoclonal Antibody [A7C4]
Antibody Type
Mouse Monoclonal Antibody
Immunogen
Recombinant protein within human Galactosidase alpha aa 51-300/429.
Species Reactivity
Human
Validated Applications
WB, IHC-P
Target Molecular Weight
Predicted band size: 49 kDa
Positive Control
MCF-7 cell lysates, human adrenal gland tissue, human kidney tissue.
Conjugation
unconjugated
Clone Number
A7C4
RRID
Product Features
Form
Liquid
Concentration
2 mg/mL.(The concentration of this product may be batch-dependent)
Storage Instructions
Shipped at 4℃. Store at +4℃ short term (1-2 weeks). Store at -20℃ long term.
Storage Buffer
PBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
Isotype
IgG2b
Purification Method
Protein A affinity purified.
Application Dilution
-
WB
-
1:8,000
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IHC-P
-
1:600
Target
Function
This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties.
Background References
1. Chen SC. et. al. Crystal Structure of alpha-Galactosidase from Thermus thermophilus: Insight into Hexamer Assembly and Substrate Specificity. J Agric Food Chem. 2020 Jun
2. Fei Y. et. al. Cloning and expression of a novel alpha-galactosidase from Lactobacillus amylolyticus L6 with hydrolytic and transgalactosyl properties. PLoS One. 2020 Jul
Subcellular Location
Lysosome.
UNIPROT
Synonyms
AGAL_HUMAN antibody
Agalsidase alfa antibody
Alpha D galactosidase A antibody
Alpha D galactoside galactohydrolase 1 antibody
Alpha D galactoside galactohydrolase antibody
Alpha gal A antibody
Alpha galactosidase A antibody
Alpha-D-galactosidase A antibody
Alpha-D-galactoside galactohydrolase antibody
Alpha-galactosidase A antibody
ExpandImages
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Western blot analysis of Galactosidase alpha on MCF-7 cell lysates with Mouse anti-Galactosidase alpha antibody (HA600088) at 1/8,000 dilution.
Lysates/proteins at 10 µg/Lane.
Predicted band size: 49 kDa
Observed band size: 49/35 kDa
Exposure time: 30 seconds;
12% 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 (HA600088) at 1/8,000 dilution was used in 5% NFDM/TBST at room temperature for 2 hours. Goat Anti-Mouse IgG - HRP Secondary Antibody (HA1006) at 1:100,000 dilution was used for 1 hour at room temperature. -
Immunohistochemical analysis of paraffin-embedded human adrenal gland tissue with Mouse anti-Galactosidase alpha antibody (HA600088) 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 (HA600088) 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. -
Immunohistochemical analysis of paraffin-embedded human kidney tissue with Mouse anti-Galactosidase alpha antibody (HA600088) at 1/600 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 (HA600088) at 1/600 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.
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"
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