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GAA Antibody

CAT: 0223-56-495Size: 400 µLDry Ice: NoHazardous: No
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CAT#:0223-56-495Size:400 µL
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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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.
NCBI Gene ID
2548
Swiss Prot
P10253
Accession Number
P10253
Host
Rabbit
Reactivity
Human
Immunogen
This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.
Clonality
Polyclonal
Conjugation
Unconjugated
Type
Primary Antibodies
Field of Research
Obesity, Signal Transduction
Purification
This antibody is purified through a protein A column, followed by peptide affinity purification.
Concentration
Batch dependent
Buffer
Supplied in PBS with 0.09% (W/V) sodium azide.
Modification
None
Shipping Conditions
Blue Ice
Storage Conditions
Store at 4˚ C for three months and -20˚ C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
Calculated Molecular Weight
105 kDa
Fragment
Rabbit Ig
Symbol
GAA
NCBI Official Name
Lysosomal alpha-glucosidase
NCBI Organism
Homo sapiens
Background Reference 01
Bailey, S.D., et al. Diabetes Care 33 (10) :2250-2253 (2010)
Background Reference 02
Labrousse, P., et al. Mol. Genet. Metab. 99 (4) :379-383 (2010)
Background Reference 03
Talmud, P.J., et al. Am. J. Hum. Genet. 85 (5) :628-642 (2009)
Background Reference 04
Aoyama, Y., et al. J. Hum. Genet. 54 (11) :681-686 (2009)
Other Product Names
Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA
Tested Applications
WB, IHC-P, IHC
Protein ID
317373572
Physical Properties
Liquid

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