GAA Antibody (YA2872)
(Synonyms: Acid maltase; GAA; Glucosidase alpha acid; LYAG)
Based on 1 Customer Validation
GAA Antibody (YA2872) is a Rabbit-derived and non-conjugated IgG monoclonal antibody, targeting to GAA.
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Host:
Rabbit
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Isotype:
IgG
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Application:
WB
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Reactivity :
Human, Mouse, Rat
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Formulation:
Supplied in rabbit IgG in 10 mM PBS, pH 7.4, 150 mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
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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
|
|---|---|
| Dilution Ratio | 1:500-1:1000 |
Product Details
GAA Antibody (YA2872) is a Rabbit-derived and non-conjugated IgG monoclonal antibody, targeting to GAA.
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Host Rabbit
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Clonality Recombinant,Monoclonal
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Species ReactivityHuman, Mouse, Rat
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Observed Molecular WeightObserved band size: 85 kDa,95 kDa
Note: Due to possible protein modifications or aggregation, the molecular weight should be confirmed by actual measurement, and the predicted value is for reference only.
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Calculated Molecular Weight Predicted band size: 105 kDa
A synthesized peptide derived from human GAA aa140-210.
Endogenous
Affinity Purified
Non-conjugated
Unmodified
IgG
Product Properties
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Appearance
Solution
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Formulation
Supplied in rabbit IgG in 10 mM PBS, pH 7.4, 150 mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
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Concentration
Batch-dependent, Please check the COA for the concentration of each lot. Check Lot Concentration
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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.
Verification Images
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Western blot analysis of extracts from HepG2 (lane2, 20μg), MCF-7 (lane3, 20μg), HEK293T (lane4, 20μg) and HEK293 (lane5, 20μg) using GAA Antibody (HY-P83127). Proteins were transferred to a PVDF membrane and blocked with 5% non-fat milk in TBST for 2 hour at room temperature. The primary antibody (1/1000) and Loading control antibody (Beta Actin, HY-P80993, 1/10,000) was used in 5% non-fat milk in TBST at 4°C overnight. Goat Anti-Rabbit IgG-HRP Secondary Antibody (HY-P8001,1/10,000) was used for 1 hour at room temperature.
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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SwissProt ID
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Synonyms
Acid maltase; GAA; Glucosidase alpha acid; LYAG
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Research Field
Signal Transduction
Documentation
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Data Sheet (261 KB)
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SDS (251 KB)
- English - EN (251 KB)
- Français - FR (251 KB)
- Deutsch - DE (251 KB)
- Norwegian - NO (251 KB)
- Español - ES (251 KB)
- Swedish - SV (251 KB)
- Italian - IT (251 KB)
- Korean - KR (251 KB)
- Portuguese - PT (251 KB)
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User Guide for Antibodies (1077 KB)
[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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