Rabbit Anti-GAA Polyclonal Antibody
DATASHEET
Host:Rabbit
Target Protein:GAA
IR:Immunogen Range:541-640/952
Clonality:Polyclonal
Isotype:IgG
Entrez Gene:2548
Swiss Prot:P10253
Source:KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha:541-640/952
Purification:affinity purified by Protein A
Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
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. [provided by RefSeq, Jul 2008].
Size:50ul
Concentration:1mg/ml
Applications:ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
IF(1:200-800)
Cross Reactive Species:Human
Mouse
Rat
Dog
Pig
Horse
Sheep
僅供科研使用。不能用于人和動物治療等其它臨床診斷用!
關鍵字: GAA;70 kDa lysosomal alp;Acid alpha glucosida;Glucosidase ;Glucosidase alpha ac;Glucosidase alpha;LYAG;LYAG_HUMAN;
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