Recombinant Human alpha-Galactosidase A/GLA Protein

重组人alpha-Galactosidase A/GLA蛋白 | 重组人α-半乳糖苷酶A

agalsidase alfa | Agalsidase alpha | Agalsidase | Alpha-D-galactosidase A | alpha-D-galactoside galactohydrolase 1 | Alpha-D-galactoside galactohydrolase | alpha-gal A | alpha-galactosidase A | EC 3.2.1 | EC 3.2.1.22 | GALA | galactosidase | alpha | GLA |

rp181244-10μg

10μg

无动物源

≥90%(SDS-PAGE)

Human alpha-Galactosidase A is a homodimeric glycoprotein that can release terminal alpha-galactosyl moieties from glycolipids and glycoproteins and catalyze the hydrolysis of melibiose into galactose and glucose. It is a lysosomal enzyme and is responsible for degradation of glycolipid globotriaosylceramide (Gb3) (Gal alpha 1‑4Gal beta 1‑4Glc beta ‑ceramide). Mutations in this gene cause Fabry disease, an X-linked hereditary lysosomal storage disease with the accumulation of Gb3 in the walls of small blood vessels, nerves, dorsal root ganglia, renal glomerular and tubular epithelial cells, and cardiomyocytes. Inability to prevent the glycosphingolipid deposition can cause hypertension, strokes, heart attack and progressive renal failure. Current treatment for Fabry disease is enzyme replacement therapy using intravenously delivered recombinant alpha-Galactosidase A.

Testing in progress

重组表达

46.2 kDa

C-His

45.0-55.0 kDa, under reducing conditions; 45.0-55.0 kDa, under non-reducing conditions.

CHO Accession #: P06280 | CHO

<1.0 EU/μg

人(Human)

1-429 aa

MQLRNPELHLGCALALRFLALVSWDIPGARALDNGLARTPTMGWLHWERFMCNLDCQEEPDSCISEKLFMEMAELMVSEGWKDAGYEYLCIDDCWMAPQRDSEGRLQADPQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFGYYDIDAQTFADWGVDLLKFDGCYCDSLENLADGYKHMSLALNRTGRSIVYSCEWPLYMWPFQKPNYTEIRQYCNHWRNFADIDDSWKSIKSILDWTSFNQERIVDVAGPGGWNDPDMLVIGNFGLSWNQQVTQMALWAIMAAPLFMSNDLRHISPQAKALLQDKDVIAINQDPLGKQGYQLRQGDNFEVWERPLSGLAWAVAMINRQEIGGPRSYTIAVASLGKGVACNPACFITQLLPVKRKLGFYEWTSRLRSHINPTGTVLLQLENTMQMSLKDLLHHHHHH

Yes

Yes