Galactosidase Alpha (GLa)
GALA; GL-A
 HGNC 4296
 MGI 1347344
 OMIM 301500
 RGD 1589721
 UCSC uc004ehl.1
 UniProt P06280
 USCN 90183
Galactosidase Alpha (GLa)
Alpha-galactosidase is a glycoside hydrolase enzyme that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. It is encoded by the GLA gene. This enzyme is a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. It 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's disease, a rare lysosomal storage disorder and sphingolipidosis that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties. Genzyme produces synthetic agalsidase beta under the brand name Fabrazyme for treatment of Fabry's disease.
Research reagent products of Galactosidase Alpha (GLa)
Organism
Protein
Antibody
ELISA Kit
CLIA Kit
Catalog Manual McAb PcAb Catalog Manual Catalog Manual
Homo sapiens (Human)rP90183Hu02PDFmA90183Hu22pA90183Hu81 sE90183HuPDFsC90183HuPDF
Mus musculus (Mouse)rP90183Mu01n/an/apA90183Mu01 sE90183Mun/an/an/a
Rattus norvegicus (Rat)n/an/an/an/a n/an/an/an/a