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Product Details
MUB0707P
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human
Host/Isotype
Mouse
/ IgG2a
Class
Monoclonal
Type
Antibody
Clone
43G7
Immunogen
alpha-Glucosidase
Conjugate
Form
Liquid
Concentration
1 mg/mL
Purification
Protein A/G
Storage buffer
PBS
Contains
0.09% sodium azide
Storage conditions
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Shipping conditions
Ambient (domestic); Wet ice (international)
Product Specific Information
reacts with the two major bands with apparent molecular weights of 76.000 and 70.000, the band with a molecular weight of about 94.000 and minor bands with apparent molecular weights of less than 67.000, when analyzing a-glucosidase isolated from placenta by polyacrylamide gel electrophoresis in the presence of SDS and a reducing agent.
The antibody may be stored at 4°C. For prolonged storage prepare appropriate aliquots and store at or below -20°C. Prior to use, an aliquot is thawed slowly in the dark at ambient temperature, spun down again and used to prepare working dilutions by adding sterile phosphate buffered saline (PBS, pH 7.2). Repeated thawing and freezing should be avoided. Working dilutions should be stored at 4°C, not refrozen, and preferably used the same day. If a slight precipitation occurs upon storage, this should be removed by centrifugation. It will not affect the performance or the concentration of the product.
Target Information
This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: Acid maltase; Aglucosidase alfa; Lysosomal alpha-glucosidase
Gene Aliases: GAA; LYAG
UniProt ID: (Human) P10253
Entrez Gene ID: (Human) 2548
maltose metabolic process
regulation of the force of heart contraction
diaphragm contraction
heart morphogenesis
glycogen catabolic process
sucrose metabolic process
glucose metabolic process
lysosome organization
locomotory behavior
tissue development
vacuolar sequestering
muscle cell cellular homeostasis
neuromuscular process controlling posture
neuromuscular process controlling balance
cardiac muscle contraction
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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