GAA Rabbit Polyclonal Antibody

SKU
TA334653
Rabbit Polyclonal Anti-GAA Antibody
  $585.00
5 Days*
Bulk/Customize
Specifications
Specifications
Product Data
Application IHC, WB
Recommended Dilution WB
Reactivity Human
Antibody Host Rabbit
Isotype IgG
Clonality Polyclonal
Immunogen The immunogen for anti-GAA antibody: synthetic peptide directed towards the N terminal of human GAA. Synthetic peptide located within the following region: FGVIVRRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPL
Buffer Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Note that this product is shipped as lyophilized powder to China customers.
Purification Affinity Purified
Conjugation Unconjugated
Storage Store at -20°C as received.
Stability Stable for 12 months from date of receipt.
Shipping Blue Ice
Predicted Protein Size 98 kDa
Gene Name glucosidase alpha, acid
Database Link
Background GAA is 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. 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.
Synonyms LYAG
Note Immunogen Sequence Homology: Dog: 100%; Pig: 100%; Rat: 100%; Human: 100%; Mouse: 100%; Bovine: 100%; Guinea pig: 100%; Horse: 93%; Zebrafish: 83%; Rabbit: 79%
Reference Data
Protein Categories Membrane Proteins
Protein Families Druggable Genome, Transmembrane
Protein Pathways Galactose metabolism, Lysosome, Metabolic pathways, Starch and sucrose metabolism
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