GLA antibody

Catalog Number:

38284

Amount:

100μg

Price:

$319

Swiss-Prot No:

Swiss-Prot#: P06280
NCBI Gene ID: 2717

Form of Antibody:

Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.

Storage/Stability:

Immunogen:

Recombinant protein of human GLA.

Purification:

Antibodies were purified by affinity purification using immunogen.

Specificity/Sensitivity:

The antibody detects endogenous level of total GLA antibody.

Applications:

WB,IHC,IF

Background:

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.

References:

appl_detail:

WB 1:500 - 1:2000
IHC 1:50 - 1:200
IF 1:50 - 1:100

Datasheet PDF