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.

Specification

• 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: LDNGLARTPTMGWLHWERFMCNLDC QEEPDSCISEKLFMEMAELMVSEGW KDAGYEYLCIDDCWMAPQRDS EGRLQADPQRFPHGIRQLANYVHSK GLKLGIYADVGNKTCAGFPGSFGYY DIDAQTFADWGVDLLKFDGCY CDSLENLADGYKHMSLALNRTGRSI VYSCEWPLYMWPFQKPNYTEIRQYC NHWRNFADIDDSWKSIKSILD WTSFNQERIVDVAGPGGWNDPDMLV IGNFGLSWNQQVTQMALWAIMAAPL FMSNDLRHISPQAKALLQDKD VIAINQDPLGKQGYQLRQGDNFEVW ERPLSGLAWAVAMINRQEIGGPRSY TIAVASLGKGVACNPACFITQ LLPVKRKLGFYEWTSRLRSHINPTG TVLLQLENTMQMSLKDLL
• 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 Information

• 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