Technical Data
G2035-48
GLA, NT (Alpha-galactosidase A, Alpha-D-galactoside Galactohydrolase, Alpha-D-galactosidase A, Agalsidase, Melibiase)
Description:
GLA 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.

Applications:
Suitable for use in ELISA, Flow Cytometry, Immunocytochemistry/Immunofluorescence, Immunohistochemistry and Western Blot. Other applications not tested.

Recommended Dilution:
ELISA: 1:1,000
Western Blot: 1:1,000
Immuncytochemistry/Immunofluorescence: 1:10 -1:50
Immunohistochemistry (paraffin): 1:10 -1:50
Optimal dilutions to be determined by the researcher.

Storage and Stability:
May be stored at 4C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
TypeIsotypeCloneGrade
PabIgGPurified
SizeStorageShippingSourceHost
200ul-20CBlue IceHumanRabbit
Concentration:
As reported
Immunogen:
KLH-conjugated synthetic peptide mapping to a fragment of residues within aa 83 -112 in the N-terminal region of human GLA, UniPot Accession # P06280
Purity:
Purified by ammonium sulfate precipitation.
Form
Supplied as a liquid in PBS, 0.09% sodium azide.
Specificity:
Recognizes human GLA.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
1. Mignani,R., Kidney Int. 75(10), 1115-1116 (2009). 2. Ioannou,Y.A., Biochem. J. 332(PT 3), 789-797 (1998).