"GLA" Related Products

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Recombinant Human GLA therapeutic protein

Cat.No.: GLA-P027H
Product Overview: Recombinant human alpha-galactosidase A. The mature protein is composed of 2 subunits of 398 residues. Protein is glycosylated and produced by CHO cells.
Description: 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. The expression product is the active ingredient of Fabrazyme and Replagal.
Source: CHO
Species: Human
Molecular Mass: 45.4 kDa
Protein length: 398 Aa
AA Sequence: LDNGLARTPTMGWLHWERFMCNLDCQEEPDSCISEKLFMEMAELMVSEGWKDAGYEYLCIDDCWMAPQRDS EGRLQADPQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFGYYDIDAQTFADWGVDLLKFDGCY CDSLENLADGYKHMSLALNRTGRSIVYSCEWPLYMWPFQKPNYTEIRQYCNHWRNFADIDDSWKSIKSILD WTSFNQERIVDVAGPGGWNDPDMLVIGNFGLSWNQQVTQMALWAIMAAPLFMSNDLRHISPQAKALLQDKD VIAINQDPLGKQGYQLRQGDNFEVWERPLSGLAWAVAMINRQEIGGPRSYTIAVASLGKGVACNPACFITQ LLPVKRKLGFYEWTSRLRSHINPTGTVLLQLENTMQMSLKDLL
Endotoxin: < 1.0 EU per μg of the protein
Purity: >95%
Storage: Can be stored at +4 centigrade short term (1-2weeks). For long term storage, aliquot and store at -20 centigrade or -70 centigrade. Avoidrepeated freezing and thawing cycles.
Gene Name: GLA galactosidase, alpha [ Homo sapiens ]
Official Symbol: GLA
Synonyms: GLA; galactosidase, alpha; alpha-galactosidase A; GALA; melibiase; alpha-gal A; agalsidase alfa; alpha-D-galactosidase A; alpha-D-galactoside galactohydrolase 1;
Gene ID: 2717
mRNA Refseq: NM_000169
Protein Refseq: NP_000160
MIM: 300644
UniProt ID: P06280
Chromosome Location: Xq21.3-q22
Pathway: Galactose metabolism, organism-specific biosystem; Galactose metabolism, conserved biosystem; Glycerolipid metabolism, organism-specific biosystem; Glycerolipid metabolism, conserved biosystem; Glycosphingolipid biosynthesis - globo series, organism-specific biosystem; Glycosphingolipid biosynthesis - globo series, conserved biosystem; Glycosphingolipid metabolism, organism-specific biosystem;
Function: alpha-galactosidase activity; alpha-galactosidase activity; alpha-galactosidase activity; catalytic activity; cation binding; galactoside binding; hydrolase activity; hydrolase activity, hydrolyzing O-glycosyl compounds; protein binding; protein homodimerization activity; raffinose alpha-galactosidase activity; receptor binding;

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