GLA Recombinant Rabbit Monoclonal Antibody [PSH25-93] - BSA and Azide free
Catalog# HA752005
GLA Recombinant Rabbit Monoclonal Antibody [PSH25-93] - BSA and Azide free
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WB
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IF-Cell
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IHC-P
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FC
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Human
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HA724585
含抗保成分
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unconjugated
Overview
Product Name
GLA Recombinant Rabbit Monoclonal Antibody [PSH25-93] - BSA and Azide free
Antibody Type
Recombinant Rabbit monoclonal Antibody
Immunogen
Recombinant protein within human GLA aa 1-429.
Species Reactivity
Human
Validated Applications
WB, IF-Cell, IHC-P, FC
Target Molecular Weight
Predicted band size: 49 kDa
Positive Control
MCF7 (Human breast cancer cell) cell lysate, HeLa (Human cervical adenocarcinoma cell) cell lysate, A431 (Human epidermoid carcinoma skin squamous cell) cell lysate, HEK-293 (Human embryonic kidney cell) cell lysate, Hep G2 (Human hepatocellular carcinoma cell) cell lysate, LNCaP (Human prostate cancer cell) cell lysate.
Conjugation
unconjugated
Clone Number
PSH25-93
Product Features
Form
Liquid
Concentration
1 mg/mL.(The concentration of this product may be batch-dependent)
Storage Instructions
Store at +4℃ after thawing. Aliquot store at -20℃. Avoid repeated freeze / thaw cycles.
Storage Buffer
PBS (pH7.4).
Isotype
IgG
Purification Method
Protein A affinity purified.
Application Dilution
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WB
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1:5,000
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IF-Cell
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1:100
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IHC-P
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1:200
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FC
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1:1,000
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 GLA on different lysates with Rabbit anti-GLA antibody (HA752005) at 1/5,000 dilution.
Lane 1: MCF7 (Human breast cancer cell) cell lysate
Lane 2: HeLa (Human cervical adenocarcinoma cell) cell lysate
Lane 3: A431 (Human epidermoid carcinoma skin squamous cell) cell lysate
Lane 4: HEK-293 (Human embryonic kidney cell) cell lysate
Lane 5: Hep G2 (Human hepatocellular carcinoma cell) cell lysate
Lane 6: LNCaP (Human prostate cancer cell) cell lysate
Lysates/proteins at 15 µg/Lane.
Exposure time: 10 seconds; ECL: K1801
Blocking: 5% NFDM/TBST, 1 hour at room temperature
Primary antibody: HA752005, 1/5,000 in primary antibody dilution buffer (K1803), overnight at 4 °C
Secondary antibody: Goat anti-Rabbit IgG-HRP (HA1001), 1/50,000 in 5% NFDM/TBST, 1 hour at room temperature
Predicted band size: 49 kDa
Observed band size: 49 kDa -
Application: Immunocytochemistry (IF-cell)
Species: Human
Sample: MCF7 (Human breast cancer cell)
Fixation: 4% Paraformaldehyde, 15 minutes at room temperature.
Permeabilization: 0.1% Triton X-100, 15 minutes at room temperature.
Blocking: 1% BSA + 10% normal goat serum, 1 hour at room temperature.
Antibody dilution buffer: 1% BSA in PBST.
Primary antibody: HA752005, 1/100, overnight at 4°C.
Secondary antibody: Goat Anti-Rabbit IgG (iFluor™ 488, HA1121), 45 minutes at room temperature.
Counterstain: Beta tubulin (HA601187, Red), 1/100, overnight at 4℃. The nuclear counterstain was DAPI (Blue). -
Application: Immunohistochemistry (IHC-P)
Species: Human
Tissue: Kidney
Sample: Paraffin-embedded section
Antigen retrieval: Heat-mediated, Tris-EDTA buffer (pH 9.0), 20 minutes at 95℃.
Wash buffer: 1× TBST
Endogenous peroxidase blocking: 3% H₂O₂, 10 minutes at room temperature.
Blocking: 1% BSA + 10% normal goat serum, 10 minutes at room temperature.
Primary antibody: HA752005, 1/200, 1 hour at room temperature.
Secondary antibody: HA1119, 20 minutes at room temperature. -
Application: Immunohistochemistry (IHC-P)
Species: Human
Tissue: Liver
Sample: Paraffin-embedded section
Antigen retrieval: Heat-mediated, Tris-EDTA buffer (pH 9.0), 20 minutes at 95℃.
Wash buffer: 1× TBST
Endogenous peroxidase blocking: 3% H₂O₂, 10 minutes at room temperature.
Blocking: 1% BSA + 10% normal goat serum, 10 minutes at room temperature.
Primary antibody: HA752005, 1/200, 1 hour at room temperature.
Secondary antibody: HA1119, 20 minutes at room temperature. -
Application: Flow Cytometry (Intra)
Species: Human
Sample: MCF7 (Human breast cancer cells)
Fixation: 4% Paraformaldehyde, 15 minutes at room temperature.
Permeabilization: 0.1% Tween-20, 15 minutes at room temperature.
Blocking: 1% BSA + 10% normal goat serum, 15 minutes at room temperature.
Antibody dilution buffer: 1x PBS.
Primary antibody: HA752005 (1/1,000, Red) compared with Rabbit IgG Isotype Control (HA722127, Green), 15 minutes at room temperature.
Secondary antibody: Goat Anti-Rabbit IgG (iFluor™ 488, HA1121), 15 minutes at room temperature.
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"