Lysosomal Alpha Glucosidase (GAA) Antibody

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.

Target	Lysosomal Alpha Glucosidase (GAA)
Clonality	Polyclonal
Reactivity	Human
Tested Applications	ELISA, WB, IHC
Host	Rabbit
Recommended dilutions	WB: 1/1000, IHC-P: 1/500. Not tested in IHC-F. Optimal dilutions/concentrations should be determined by the end user.
Conjugation	Unconjugated
Immunogen	KLH-conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.
Isotype	IgG
Form	Liquid
Purification	Purified through a protein A column, followed by peptide affinity purification.
Storage	Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
UniProt Primary AC	P10253 (UniProt, ExPASy)
UniProt Secondary AC	Q09GN4, Q14351, Q16302, Q8IWE7
UniProt Entry Name	LYAG_HUMAN
KEGG	hsa:2548
String	9606.ENSP00000305692
Molecular Weight	Calculated MW: 105 kDa
Buffer	PBS containing 0.09% sodium azide.
Availability	Shipped within 5-10 working days.
Note	This product is for research use only.

Research Articles on Lysosomal Alpha Glucosidase (GAA)

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