Anti-Galactosidase alpha/Gla Antibody Picoband®, Dylight594 Conjugate

About this product

Boster Bio Anti-Galactosidase alpha/Gla Antibody Picoband® catalog # A01135-2. Tested in IHC, WB applications. This antibody reacts with Human. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Specifications

Catalog number

A01135-2-Dylight594

Size / count

100 µg

Dry ice

No

Hazardous

No

Discontinued

Yes

Background

Alpha-galactosidase is a glycoside hydrolase enzyme that encoded by the GLA gene. This gene is 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.

Synonyms

Alpha-galactosidase A;3.2.1.22 ;Alpha-D-galactosidase A;Alpha-D-galactoside galactohydrolase;Melibiase;Gla;Ags;

Gene Name

Alpha-galactosidase A

Gene ID

2717

UniProt

P06280

Host

Rabbit

Reactivity

Human

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

A synthetic peptide corresponding to a sequence at the C-terminus of human Galactosidase alpha/Gla.

Clonality

Polyclonal

Applications

WB,IHC

Purification

Immunogen affinity purified.

Concentration

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Form

Lyophilized

Reconstitution

Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Function

Factor D cleaves factor B when the latter is complexed with factor C3b, activating the C3bbb complex, which then becomes the C3 convertase of the alternate pathway. Its function is homologous to that of C1s in the classical pathway.

Components

Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.01mg NaN3.

References & Citations

1. "Entrez Gene: GLA galactosidase, alpha". 2. Calhoun DH, Bishop DF, Bernstein HS, Quinn M, Hantzopoulos P, Desnick RJ (1985). "Fabry disease: isolation of a cDNA clone encoding human alpha-galactosidase A". Proceedings of the National Academy of Sciences of the United States of America. 82 (21): 7364–8. 3. Keating GM (October 2012). "Agalsidase alfa: a review of its use in the management of Fabry disease". BioDrugs. 26 (5): 335–54.

Storage Conditions

Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.

Calculated Molecular Weight

47643 MW

Observed Molecular Weight

49 kDa

Fragment

Rabbit IgG

Applications Notes

Western blot, 0.25-0.5μg/ml, Human<br> Immunohistochemistry (Paraffin-embedded Section), 2-5μg/ml, Human<br>

Subcellular Location

Lysosome.

Protein Name

Alpha-galactosidase A