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Galactosidase alpha Mouse Monoclonal Antibody [A7C4]

Usd: 350

Specification

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Catalog# HA600088

Galactosidase alpha Mouse Monoclonal Antibody [A7C4]

Application

  • WB

  • IHC-P

Reactivity

  • Human

Conjugation

  • 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

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

AB_3071704

Product Features

Form

Liquid

Concentration

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

  • 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

SwissProt: P06280 Human

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

Expand

Images

  • Western blot analysis of Galactosidase alpha on MCF-7 cell lysates with Mouse anti-Galactosidase alpha antibody (<a href="/products/HA600088" style="font-weight: bold;text-decoration: underline;">HA600088</a>) at 1/8,000 dilution.<br /><br />Lysates/proteins at 10 µg/Lane.<br /><br />Predicted band size: 49 kDa<br />Observed band size: 49/35 kDa<br /><br />Exposure time: 30 seconds;<br /><br />12% SDS-PAGE gel.<br /><br />Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (<a href="/products/HA600088" style="font-weight: bold;text-decoration: underline;">HA600088</a>) at 1/8,000 dilution was used in 5% NFDM/TBST at room temperature for 2 hours. Goat Anti-Mouse IgG - HRP Secondary Antibody (<a href="/products/HA1006" style="font-weight: bold;text-decoration: underline;">HA1006</a>) at 1:100,000 dilution was used for 1 hour at room temperature.

    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 (<a href="/products/HA600088" style="font-weight: bold;text-decoration: underline;">HA600088</a>) at 1/1,000 dilution.<br /><br />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 ddH<sub>2</sub>O and PBS, and then probed with the primary antibody (<a href="/products/HA600088" style="font-weight: bold;text-decoration: underline;">HA600088</a>) 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 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 (<a href="/products/HA600088" style="font-weight: bold;text-decoration: underline;">HA600088</a>) at 1/600 dilution.<br /><br />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 ddH<sub>2</sub>O and PBS, and then probed with the primary antibody (<a href="/products/HA600088" style="font-weight: bold;text-decoration: underline;">HA600088</a>) 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.

    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"