Product Datasheet
GLA antibody
Catalog Number: 38284
Technical:tech@swbio.com
Information:info@swbio.com
Description
- 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.
- Immunogen:
- Recombinant protein of human GLA.
- appl_detail:
- WB 1:500 - 1:2000
IHC 1:50 - 1:200
IF 1:50 - 1:100
- Purification:
- Antibodies were purified by affinity purification using immunogen.
- Specificity:
- The antibody detects endogenous level of total GLA antibody.
- 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.
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