GLA/alpha-Galactosidase A Active Protein | GLA active protein

GLA/alpha-Galactosidase A Protein, Mouse, Recombinant (His Tag)

Cat. Num. AAA8123062

• Gene Names: Gla; Ags
• Purity: >95% as determined by SDS-PAGE
• Synonyms: GLA/alpha-Galactosidase A; GLA/alpha-Galactosidase A Protein; Mouse; Recombinant (His Tag); Mouse alpha-Galactosidase A/GLA Protein (His Tag); Ags Protein; galactosidase; alpha; GLA active protein

• Host: HEK293 Cells
• Purity/Purification: >95% as determined by SDS-PAGE
• Form/Format: Lyophilized from sterile PBS, pH7.4. Normally 5%-8% trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization. Please refer to the specific buffer information in the hard copy of CoA.
• Sequence: Met1-Arg421

• Species: Mouse
• Activity: Measured by its ability to hydrolyze 4-methylumbelliferyl-alpha-D-galactopyranoside. The specific activity is >400pmoles/min/ug.
• Endotoxin: <1.0EU per ug of the protein as determined by the LAL method
• Predicted N Terminal: Leu 34
• Tag: C-His
• Protein Construction: A DNA sequence encoding the mouse Gla (Q8BGZ6)(Met1-Arg421) was expressed with a C-terminal polyhistidine tag.
• Reconstitution: A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.
• Preparation and Storage: Samples are stable for up to twelve months from date of receipt at -20 degree C to -80 degree C. Store it under sterile conditions at -20 degree C to -80 degree C. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.; In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature. Bulk packages of recombinant proteins are provided as frozen liquid.; They are shipped out with blue ice.

SDS-Page

Related Product Information for GLA active protein

Background: Alpha-galactosidase A, also known as Alpha-D-galactoside galactohydrolase, Alpha-D-galactosidase A, Melibiase and GLA, is a member of the glycosyl hydrolase 27 family. GLA is used as a long-term enzyme replacement therapy in patients with a confirmed test of Fabry disease. Defects in GLA are the cause of Fabry disease (FD) which 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. Deficiency of GLA leads to the accumulation of glycosphingolipids in the vasculature leading to multiorgan pathology. In addition to well-described microvascular disease, deficiency of GLA is also characterized by premature macrovascular events such as stroke and possibly myocardial infarction.

References

Koide T.et al., 1990, FEBS Lett. 259:353-356. Yang C.-C. et al., 2003, Clin. Genet. 63:205-209. Verovnik F. et al.,2004, Eur. J. Hum. Genet. 12:678-681. Nance C.S. et al., 2006, Arch. Neurol. 63:453-457.

NCBI and Uniprot Product Information

• NCBI GI #: 133778924
• NCBI GeneID: 11605
• NCBI Accession #: NP_038491.2
• NCBI GenBank Nucleotide #: NM_013463.2
• UniProt Accession #: Q8BGZ6; P51569
• Molecular Weight: 47,845 Da
• NCBI Official Full Name: alpha-galactosidase A
• NCBI Official Synonym Full Names: galactosidase, alpha
• NCBI Official Symbol: Gla
• NCBI Official Synonym Symbols: Ags
• NCBI Protein Information: alpha-galactosidase A
• UniProt Protein Name: Alpha-galactosidase A
• Protein Family: Glycerol facilitator-aquaporin
• UniProt Gene Name: Gla