GAA Antibody
Product Specifications
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.
NCBI Gene ID
2548
Swiss Prot
P10253
Host
Rabbit
Reactivity
Human, Mouse, Rat
Immunogen
A synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_000143.2) .
Clonality
Polyclonal
Conjugation
Unconjugated
Type
Primary Antibodies
Field of Research
Cancer, Signal Transduction
Purification
Affinity purification
Positive Control
LO2
Concentration
Batch dependent
Buffer
PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Modification
None
Shipping Conditions
Blue Ice
Storage Conditions
Store at -20˚ C. Avoid freeze / thaw cycles.
Calculated Molecular Weight
Observed: 105kDa
Fragment
IgG
Symbol
GAA
Positive Control 2
HT-29
Positive Control 3
293T
Positive Control 4
A-549
Positive Control 5
DU145
Positive Control 6
Mouse liver
NCBI Official Name
Lysosomal alpha-glucosidase
NCBI Organism
Homo sapiens
Other Product Names
Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA
Tested Applications
WB, IF, IP
Physical Properties
Liquid
Curated Selection
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