GAA Antibody (YA9716)
(Synonyms: 70 kDa lysosomal alpha-glucosidase, Acid alpha glucosidase, Acid maltase, Aglucosidase alfa, Alpha glucosidase, GAA, Glucosidase alpha acid (Pompe disease glycogen storage disease type II), Glucosidase alpha acid, Glucosidase alpha, LYAG)GAA Antibody (YA9716) is a Rabbit-derived and non-conjugated IgG Recombinant,Monoclonal antibody, targeting to GAA.
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Host:
Rabbit
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Isotype:
IgG
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Application:
WB, IF-Tissue, IHC-P
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Reactivity :
Human
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Formulation:
Supplied in TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
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Conjugation:
Non-conjugated
To place orders, for customer services and technical support, please contact: MedChemExpress USA
Tel: 609-228-6898 E-mail: [email protected] [email protected]
Applications
| Application |
WB
WB: Western Blot
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IF-Tissue
IF-Tissue: Immunofluorescence-Tissue
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IHC-P
IHC-P: Immunohistochemistry-Paraffin
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|---|---|---|---|
| Dilution Ratio | 1:500-2000 | 1:50-200 | 1:50-1000 |
Product Details
GAA Antibody (YA9716) is a Rabbit-derived and non-conjugated IgG Recombinant,Monoclonal antibody, targeting to GAA.
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Host Rabbit
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Clonality Recombinant,Monoclonal
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Species ReactivityHuman
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Calculated Molecular Weight Predicted band size: 105/76/70 kDa;
Recombinant protein of Human G(aa (aa 120-230).
Endogenous
affinity purified.
Non-conjugated
Unmodified
IgG
Product Properties
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Appearance
Solution
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Formulation
Supplied in TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
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Storage & Stability
Stored at -20°C for 1 year. Avoid repeated freeze / thaw cycles.
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Shipping
Shipping with blue ice.
Background
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Function
GAA is 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][2][3][4][5].
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Subcellular Localization
Lysosome; Lysosome membrane
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Isoforms & Post-Translational Modification
GAA has an amino acid length of 952, molecular weight is 105324 Da.
The different forms of acid glucosidase are obtained by proteolytic processing. -
SwissProt ID
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Synonyms
70 kDa lysosomal alpha-glucosidase, Acid alpha glucosidase, Acid maltase, Aglucosidase alfa, Alpha glucosidase, GAA, Glucosidase alpha acid (Pompe disease glycogen storage disease type II), Glucosidase alpha acid, Glucosidase alpha, LYAG
Documentation
References
[1]. Hermans MM, et al. Twenty-two novel mutations in the lysosomal alpha-glucosidase gene (GAA) underscore the genotype-phenotype correlation in glycogen storage disease type II. Hum Mutat. 2004 Jan;23(1):47-56. [Content Brief]
[2]. Pittis MG, et al. Molecular and functional characterization of eight novel GAA mutations in Italian infants with Pompe disease. Hum Mutat. 2008 Jun;29(6):E27-36. [Content Brief]
[3]. Hermans MM, et al. Human lysosomal alpha-glucosidase. Characterization of the catalytic site. J Biol Chem. 1991 Jul 25;266(21):13507-12. [Content Brief]
[4]. Boerkoel CF, et al. Leaky splicing mutation in the acid maltase gene is associated with delayed onset of glycogenosis type II. Am J Hum Genet. 1995 Apr;56(4):887-97. [Content Brief]
[5]. Roig-Zamboni V, et al. Structure of human lysosomal acid α-glucosidase-a guide for the treatment of Pompe disease. Nat Commun. 2017 Oct 24;8(1):1111. [Content Brief]
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