Anti-Galactosidase alpha/Gla Antibody Picoband® Fluoro647 Conjugated
Product Specifications
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
GLA
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
Flow Cytometry
Field of Research
Mesenchymal Stem Cells, Stem Cells
Purification
Immunogen affinity purified.
Form
Liquid
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.
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
At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.
Calculated Molecular Weight
47643 MW
Applications Notes
6
Gene Name Synonym
Alpha-galactosidase A
Subcellular Location
Lysosome.
Protein Name
Alpha-galactosidase A
Isotype
Rabbit IgG
Contents
Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.
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