Research Grade Myozyme/Alglucosidase Alfa (HY377296)

Name: Research Grade Myozyme/Alglucosidase Alfa
Brand: abinScience
SKU: HY377296
Price: 192 USD
Availability: InStock

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Overview

Catalog No.	HY377296
Species reactivity	Human
Applications	ELISA, Functional assay, Research in vivo
Biological activity	Lysosomal α-Glucosidase is a γ-amylase that is essential for the degradation of glycogen in lysosomes [1] [2].
Expression system	Mammalian cells
Clonality	Monoclonal
Target	GAA, Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa
Endotoxin level	Please contact the lab for this information.
Purity	>95% purity as determined by SDS-PAGE.
Purification	Purified by ion exchange chromatography.
Accession	P10253
Form	Lyophilized
Storage buffer	Lyophilized from PBS (pH 7.4), 1 mM EDTA, 4% trehalose, 1% mannitol. Please refer to the specific buffer information in the hardcopy of datasheet or the lot-specific COA.
Stability and Storage	Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt.
Background	Lysosomal alpha-glucosidase (GAA/Aglucosidase alfa) is a ~105 kDa protein. Essential for the degradation of glycogen in lysosomes. Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linked glucans. 1. Hermans, MM. et al. (2004) Human mutation 23, 47-56. PMID: 14695532 2. Pittis, MG. et al. (2008) Human mutation 29, E27-36. PMID: 18429042 3. Hermans, MM. et al. (1991) The Journal of biological chemistry 266, 13507-12. PMID: 1856189 4. Boerkoel, CF. et al. (1995) American journal of human genetics 56, 887-97. PMID: 7717400 5. Roig-Zamboni, V. et al. (2017) Nature communications 8, 1111. PMID: 29061980
References	1. Hermans MM, et al. Human lysosomal alpha-glucosidase: functional characterization of the glycosylation sites. The Biochemical journal. 1993 Feb 01;289 (Pt 3)(Pt 3):681-6. [HY377296] 2. Hoefsloot LH, et al. Primary structure and processing of lysosomal alpha-glucosidase; homology with the intestinal sucrase-isomaltase complex. The EMBO journal. 1988 Jun;7(6):1697-704. [HY377296]
Note	For research use only. Not suitable for clinical or therapeutic use.