GAA (N-term) Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IHC, WB
Recommend Dilution	ELISA: 1/1000. Western Blot: 1/100-1/500. Immunohistochemistry on Paraffin Sections: 1/10-1/50.
Reactivity	Human
Host	Rabbit
Clonality	Polyclonal
Immunogen	conjugated synthetic peptide between 173-203 amino acids from the N-terminal region of Human Alpha-glucosidase
Specificity	This antibody recognizes Human Alpha-glucosidase (N-term).
Formulation	PBS containing 0.09% (W/V) Sodium Azide as preservative State: Aff - Purified State: Liquid purified Ig fraction
Concentration	lot specific
Purification	Protein A column, followed by peptide affinity purification
Conjugation	Unconjugated
Storage Condition	Store undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing.
Gene Name	glucosidase alpha, acid
Database Link	Entrez Gene 2548 Human UniProt ID P10253
Background	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	Acid maltase, Aglucosidase alfa, GAA
Note	Molecular Weight: 105319 Da
Reference Data
Protein Families	Druggable Genome, Transmembrane
Protein Pathways	Galactose metabolism, Lysosome, Metabolic pathways, Starch and sucrose metabolism

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