Recombinant Human GLA Protein, His-tagged

• Cat.No.: GLA-380H
• Product Overview: Recombinant human GLA, fused to His-tag at C-terminus, was expressed in insect cell and purified by using conventional chromatography techniques.

Specification

• Description: GLA, also known as alpha-galactosidase A, is homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. 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.
• Form: Liquid. In Phosphate Buffered Saline (pH 7.4) containing 10% glycerol.
• Molecular Mass: 46.4kDa (406aa)40-57KDa (SDS-PAGE under reducing conditions.)
• AA Sequence: LDNGLARTPT MGWLHWERFM CNLDCQEEPD SCISEKLFME MAELMVSEGW KDAGYEYLCI DDCWMAPQRD SEGRLQADPQ RFPHGIRQLANYVHSKGLKL GIYADVGNKT CAGFPGSFGY YDIDAQTFAD WGVDLLKFDG CYCDSLENLA DGYKHMSLAL NRTGRSIVYS CEWPLYMWPFQKPNYTEIRQ YCNHWRNFAD IDDSWKSIKS ILDWTSFNQE RIVDVAGPGG WNDPDMLVIG NFGLSWNQQV TQMALWAIMA APLFMSNDLRHISPQAKALL QDKDVIAINQ DPLGKQGYQL RQGDNFEVWE RPLSGLAWAV AMINRQEIGG PRSYTIAVAS LGKGVACNPA CFITQLLPVK RKLGFYEWTS RLRSHINPTG TVLLQLENTM QMSLKDLLVE HHHHHH
• Endotoxin: < 1.0 EU per 1 microgram of protein (determined by LAL method)
• Purity: >90% by SDS - PAGE
• Storage: Can be stored at +4 centigrade short term (1-2 weeks). For long term storage, aliquot and store at -20 centigrade or -70 centigrade. Avoid repeated freezing and thawing cycles.
• Concentration: 0.5mg/ml (determined by Absorbance at 280nm)

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