Alpha-Galactosidase Recombinant Protein | GAL recombinant protein

Recombinant Human Alpha-Galactosidase

Cat. Num. AAA140883

• Gene Names: GLA; GALA
• Purity: Greater than 90.0% as determined by SDS-PAGE.
• Synonyms: Alpha-Galactosidase; Recombinant Human Alpha-Galactosidase; GLA Human; Alpha-Galactosidase Human Recombinant; Alpha-galactosidase A; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Melibiase; Agalsidase; GLA; GALA; GAL recombinant protein

• Host: Sf9 Baculovirus Cells
• Purity/Purification: Greater than 90.0% as determined by SDS-PAGE.
• Form/Format: GLA protein solution (0.5mg/ml) contains Phosphate Buffered Saline (pH 7.4) and 10% glycerol.; Sterile Filtered colorless solution.
• Sequence: LDNGLARTPT MGWLHWERFM CNLDCQEEPD SCISEKLFME MAELMVSEGWKDAGYEYLCI DDCWMAPQRD SEGRLQADPQ RFPHGIRQLA NYVHSKGLKL GIYADVGNKT CAGFPGSFGYYDIDAQTFAD WGVDLLKFDG CYCDSLENLA DGYKHMSLAL NRTGRSIVYS CEWPLYMWPF QKPNYTEIRQYCNHWRNFAD IDDSWKSIKS ILDWTSFNQE RIVDVAGPGG WNDPDMLVIG NFGLSWNQQV TQMALWAIMAAPLFMSNDLR HISPQAKALL QDKDVIAINQ DPLGKQGYQL RQGDNFEVWE RPLSGLAWAV AMINRQEIGGPRSYTIAVAS LGKGVACNPA CFITQLLPVK RKLGFYEWTS RLRSHINPTG TVLLQLENTM QMSLKDLLVEHHHHHH.
• Sequence Length: 429

• Preparation and Storage: Store at 4 degree C if entire vial will be used within 2-4 weeks. Store, frozen at -20 degree C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Avoid multiple freeze-thaw cycles.

Related Product Information for GAL recombinant protein

Introduction: Alpha-galactosidase A (GLA) is a homodimeric glycoprotein which hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. GLA catalyzes the hydrolysis of melibiose into galactose and glucose. Various mutations in the GLA gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease (a rare lysosomal storage disorder which results from a failure to catabolize alpha-D-galactosyl glycolipid moieties).

Description: GLA produced in Sf9 Baculovirus cells is a single, glycosylated polypeptide chain containing 406 amino acids (32-429 a.a.) and having a molecular mass of 46.4kDa (Migrates at 40-57kDa on SDS-PAGE under reducing conditions).GLA is expressed with an 8 amino acid His tag at C-Terminus and purified by proprietary chromatographic techniques.

Product Categories/Family for GAL recombinant protein

Enzymes

NCBI and Uniprot Product Information

• NCBI GI #: 4504009
• NCBI GeneID: 2717
• NCBI Accession #: NP_000160.1
• NCBI GenBank Nucleotide #: NM_000169.2
• UniProt Accession #: P06280; Q6LER7
• Molecular Weight: 48,767 Da
• NCBI Official Full Name: alpha-galactosidase A
• NCBI Official Synonym Full Names: galactosidase alpha
• NCBI Official Symbol: GLA
• NCBI Official Synonym Symbols: GALA
• NCBI Protein Information: alpha-galactosidase A
• UniProt Protein Name: Alpha-galactosidase A
• Protein Family: Alpha-galactosidase
• UniProt Gene Name: GLA
• UniProt Entry Name: AGAL_HUMAN

Uniprot Description

GLA: Defects in GLA are the cause of Fabry disease (FD). FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities. Belongs to the glycosyl hydrolase 27 family.

Protein type: Carbohydrate Metabolism - galactose; EC 3.2.1.22; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Hydrolase; Lipid Metabolism - glycerolipid; Lipid Metabolism - sphingolipid

Chromosomal Location of Human Ortholog: Xq22

Cellular Component: cytoplasm; extracellular region; Golgi apparatus; lysosomal lumen; lysosome

Molecular Function: alpha-galactosidase activity; catalytic activity; hydrolase activity; protein binding; protein homodimerization activity; receptor binding

Biological Process: glycosphingolipid catabolic process; glycosphingolipid metabolic process; glycosylceramide catabolic process; negative regulation of nitric oxide biosynthetic process; negative regulation of nitric-oxide synthase activity; oligosaccharide metabolic process

Disease: Fabry Disease