GAA recombinant monoclonal antibody, clone 5E5
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Specification
Product Description
Rabbit recombinant monoclonal antibody raised against human GAA.
Antibody Species
Rabbit
Immunogen
Original antibody is raised against a synthetic peptide corresponding to human GAA.
Reactivity
Human
Form
Liquid
Purification
Affinity chromatography purification
Isotype
IgG
Recommend Usage
ELISA
Immunohistochemistry(1:50-1:200)
The optimal working dilution should be determined by the end user.Storage Buffer
In PBS, pH7.4 (150 mM NaCl, 0.02% sodium azide and 50% glycerol)
Storage Instruction
Store at -20°C or -80°C.
Aliquot to avoid repeated freezing and thawing.Note
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Applications
Immunohistochemistry
Immunohistochemistry image of GAA recombinant monoclonal antibody, clone 5E5 diluted at 1:50 and staining in paraffin-embedded human placenta tissue performed on a Leica BondTM system.Enzyme-linked Immunoabsorbent Assay
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Gene Info — GAA
Entrez GeneID
2548Protein Accession#
P10253Gene Name
GAA
Gene Alias
LYAG
Gene Description
glucosidase, alpha; acid
Gene Ontology
HyperlinkGene Summary
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. [provided by RefSeq
Other Designations
acid alpha-glucosidase|acid maltase|alpha-glucosidase|glycogen storage disease type II|lysosomal alpha-glucosidase
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Interactome
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Pathway
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Disease
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