GAA Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	ICC/IF, IP, WB
Recommend Dilution	WB,1:500 - 1:2000 IF,1:50 - 1:200 IP,1:50 - 1:200
Reactivity	Human, Mouse, Rat
Modifications	Unmodified
Host	Rabbit
Clonality	Polyclonal
Immunogen	A synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_000143.2).
Formulation	PBS with 0.05% proclin300,50% glycerol,pH7.3.
Concentration	lot specific
Purification	Affinity purification
Conjugation	Unconjugated
Storage Condition	Store at -20℃. Avoid freeze / thaw cycles.
Predicted Protein Size	105kDa
Gene Name	glucosidase alpha, acid
Database Link	Entrez Gene 2548 Human UniProt ID P10253
Background	This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants.
Synonyms	LYAG
Reference Data

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