Recombinant Human GLA

• Cat.No.: GLA-28966TH
• Product Overview: Recombinant full length Human Galactosidase alpha with a N terminal proprietary tag; predicted MWt 72.93 kDa inclusive of tag. AAH02689

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.
• Protein length: 429 amino acids
• Molecular Weight: 72.930kDa inclusive of tags
• Source: Wheat germ
• Form: Liquid
• Purity: Proprietary Purification
• Storage buffer: pH: 8.00Constituents:0.3% Glutathione, 0.79% Tris HCl
• Storage: Shipped on dry ice. Upon delivery aliquot and store at -80oC. Avoid freeze / thaw cycles.
• Sequences of amino acids: MQLRNPELHLGCALALRFLALVSWDIPGARALDNGLARTP TMGWLHWERFMCNLDCQEEPDSCISEKLFMEMAELMVSEG WKDAGYEYLCIDDCWMAPQRDSEGRLQADPQRFPHGIRQL ANYVHSKGLKLGIYADVGNKTCAGFPGSFGYYDIDAQTFA DWGVDLLKFDGCYCDSLENLADGYKHMSLALNRTGRSIVY SCEWPLYMWPFQKPNYTEIRQYCNHWRNFADIDDSWKSIK SILDWTSFNQERIVDVAGPGGWNDPDMLVIGNFGLSWNQQ VTQMALWAIMAAPLFMSNDLRHISPQAKALLQDKDVIAIN QDPLGKQGYQLRQGDNFEVWERPLSGLAWAVAMINRQEIG GPRSYTIAVASLGKGVACNPACFITQLLPVKRKLGFYEWT SRLRSHINPTGTVLLQLENTMQMSLKDLL

Gene Information

• Gene Name: GLA galactosidase, alpha [ Homo sapiens ]
• Official Symbol: GLA
• Synonyms: GLA; galactosidase, alpha; alpha-galactosidase A; GALA
• 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
• Function: alpha-galactosidase activity; alpha-galactosidase activity; catalytic activity; cation binding; galactoside binding