GAA Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IHC, WB
Recommend Dilution	WB
Reactivity	Human
Host	Rabbit
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
Formulation	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 Condition	Store at -20°C as received.
Predicted Protein Size	98 kDa
Gene Name	glucosidase alpha, acid
Database Link	NP_000143 Entrez Gene 2548 Human UniProt ID P10253
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 Families	Druggable Genome, Transmembrane
Protein Pathways	Galactose metabolism, Lysosome, Metabolic pathways, Starch and sucrose metabolism

*Delivery time may vary from web posted schedule. Occasional delays may occur due to unforeseen complexities in the preparation of your product. International customers may expect an additional 1-2 weeks in shipping.