alpha-Galactosidase A/GLA Antibody

Immunohistochemistry-Paraffin: alpha-Galactosidase A/GLA Antibody [NBP1-89796] - Analysis in human seminal vesicle and skeletal muscle tissues. Corresponding GLA RNA-seq data are presented for the same tissues.

Immunohistochemistry-Paraffin: alpha-Galactosidase A/GLA Antibody [NBP1-89796] - Staining of human adrenal gland, colon, liver and testis using Anti-GLA antibody NBP1-89796 (A) shows similar protein distribution across tissues to independent antibody NBP1-89797 (B).

Immunohistochemistry-Paraffin: alpha-Galactosidase A/GLA Antibody [NBP1-89796] - Staining of human duodenum shows moderate positivity in lysosomes in glandular cells.

Immunohistochemistry-Paraffin: alpha-Galactosidase A/GLA Antibody [NBP1-89796] - Staining of human fallopian tube shows moderate positivity in lysosomes in glandular cells.

Immunohistochemistry-Paraffin: alpha-Galactosidase A/GLA Antibody [NBP1-89796] - Staining of human testis shows moderate positivity in lysosomes in Leydig cells.

Immunohistochemistry-Paraffin: alpha-Galactosidase A/GLA Antibody [NBP1-89796] - Staining of human skeletal muscle shows no positivity in myocytes as expected.

Immunohistochemistry-Paraffin: alpha-Galactosidase A/GLA Antibody [NBP1-89796] - Staining of human seminal vesicle shows moderate to strong positivity in lysosomes in glandular cells.

Immunohistochemistry-Paraffin: alpha-Galactosidase A/GLA Antibody [NBP1-89796] - Staining of human adrenal gland using Anti-GLA antibody.

Immunohistochemistry-Paraffin: alpha-Galactosidase A/GLA Antibody [NBP1-89796] - Staining of human colon using Anti-GLA antibody.

Immunohistochemistry-Paraffin: alpha-Galactosidase A/GLA Antibody [NBP1-89796] - Staining of human liver using Anti-GLA antibody.

• Reactivity: Hu, Mu
• Applications: IHC, IHC-P
• Clonality: Polyclonal
• Host: Rabbit
• Conjugate: Unconjugated

alpha-Galactosidase A/GLA Antibody Summary

• Immunogen: This antibody was developed against Recombinant Protein corresponding to amino acids: HISPQAKALLQDKDVIAINQDPLGKQGYQLRQGDNFEVWERPLSGLAWAVAMINRQEIGGPRSYTIAVASLGKGVACNPACFITQLLPVKRKLGFYEWTSRLRSHINPTGT
• Isotype: IgG
• Clonality: Polyclonal
• Host: Rabbit
• Gene: GLA
• Purity: Immunogen affinity purified

Applications/Dilutions

• Dilutions:
  • Immunohistochemistry 1:500 - 1:1000
  • Immunohistochemistry-Paraffin 1:500 - 1:1000
• Application Notes: For IHC-Paraffin, HIER pH 6 retrieval is recommended.
• Control Peptide:

  alpha-Galactosidase A/GLA Protein (NBP1-89796PEP)

Reactivity Notes

Mouse reactivity reported in scientific literature (PMID: 25915924).

Packaging, Storage & Formulations

• Storage: Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
• Buffer: PBS (pH 7.2) and 40% Glycerol
• Preservative: 0.02% Sodium Azide
• Purity: Immunogen affinity purified

Alternate Names for alpha-Galactosidase A/GLA Antibody

• agalsidase alfa
• Agalsidase alpha
• Agalsidase
• Alpha-D-galactosidase A
• alpha-D-galactoside galactohydrolase 1
• Alpha-D-galactoside galactohydrolase
• alpha-gal A
• alpha-galactosidase A
• EC 3.2.1
• EC 3.2.1.22
• GALA
• galactosidase, alpha
• GLA
• Melibiase

Background

Galactosidase alpha 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. [provided by RefSeq]

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Publications for alpha-Galactosidase A/GLA Antibody (NBP1-89796)(1)

Publication using NBP1-89796

• Su Xu, Yi Lun, Nastry Brignol et al. Coformulation of a Novel Human alpha-Galactosidase A With the Pharmacological Chaperone AT1001 Leads to Improved Substrate Reduction in Fabry Mice. Molecular Therapy 2015-04-27 [PMID: 25915924] (IHC, Mouse)

Bioinformatics

• Gene Symbol: GLA