Agalsidase alfa, Recombinant human alpha-galactosidase A

Catalog#	Product Name	Availability	Size	Price
THP-0115	Agalsidase alfa, Recombinant human alpha-galactosidase A	April 02, 2023	10ug	$498
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THP-0115	Agalsidase alfa, Recombinant human alpha-galactosidase A	April 02, 2023	50ug	$898
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Specification Drug Information Related Products

Cat#:	THP-0115
Product Name:	Agalsidase alfa, Recombinant human alpha-galactosidase A
Description:	Recombinant human alpha-galactosidase A, a mature protein composed of 2 subunits of 398 residues, also glycosylated and produced in CHO cells.
Sequences:	LDNGLARTPTMGWLHWERFMCNLDCQEEPDSCISEKLFMEMAELMVSEGWKDAGYEYLCIDDCWMAPQRDSEGRLQADPQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFGYYDIDAQTFADWGVDLLKFDGCYCDSLENLADGYKHMSLALNRTGRSIVYSCEWPLYMWPFQKPNYTEIRQYCNHWRNFADIDDSWKSIKSILDWTSFNQERIVDVAGPGGWNDPDMLVIGNFGLSWNQQVTQMALWAIMAAPLFMSNDLRHISPQAKALLQDKDVIAINQDPLGKQGYQLRQGDNFEVWERPLSGLAWAVAMINRQEIGGPRSYTIAVASLGKGVACNPACFITQLLPVKRKLGFYEWTSRLRSHINPTGTVLLQLENTMQMSLKDLL
Species:	Human
Molecular Weight:	45351.6 Da
Source:	CHO
Purity:	>99% by SDS-Page and HPLC analysis
Cas No:	104138-64-9
Formula:	C2029H3080N544O587S27
Endotoxin Level:	<0.001 EU per 1 μg of the protein by the LAL method
Biological Activity:	1mg/ml
Storage:	Lyophilized protein should be stored at < -20°C, though stable at room temperature for 3 weeks. Reconstituted protein solution can be stored at 2-8 °C for 1 week. Aliquots of reconstituted samples are stable at < -20°C for 3 months.

Agalsidase alfa:	Agalsidase beta
Applications:	For treatment of Fabry's disease (alpha-galactosidase A deficiency).
Examples of Clinical Use:	Fabry disease
Pharmacodynamics:	Used in the treatment of Fabry disease, an X-linked genetic disorder of glycosphingolipid metabolism. The disease is characterized by a deficiency of the lysosomal enzyme alpha-galactosidase A, which leads to progressive accumulation of glycosphingolipids, predominantly GL-3, in many body tissues. Clinical manifestations of Fabry disease include renal failure, cardiomyopathy, and cerebrovascular accidents. Recombinant human alpha-galactosidase A is intended to provide an exogenous source of alpha-galactosidase A and to limit the accumulation of these glycolipids in the tissues. Studies show unconclusive evidence for the clinical efficacy of either agalasidase alfa or agalasidase beta in preventing morbidity.
Mechanism of action:	Alpha-galactosidase A catalyzes the hydrolysis of globotriaosylceramide (GL-3) and other a-galactyl-terminated neutral glycosphingolipids, such as galabiosylceramide and blood group B substances to ceramide dihexoside and galactose.
Affected organisms:	Humans and other mammals