GAA 产品
(Glucosidase, Alpha, Acid (GAA))
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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].
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Featured GAA Categories
GAA 抗体
High quality antibodies with extensive validation data.
GAA ELISA试剂盒
Reliable ELISA kits for a wide range of species.
GAA 蛋白
Proteins for various applications incl. WB, ELISA, IF etc.
Recommended GAA 抗体
Product
Reactivity
Application
Validations
Cat. No.
Quantity
Datasheet
Reactivity
Human
Application
WB, IHC, IP, ICC
Validations
- (4)
Cat. No.
ABIN7431263
Quantity
100 μL
Datasheet
Datasheet
Reactivity
Mouse
Application
WB, IHC, IP, ICC
Validations
- (4)
Cat. No.
ABIN7439087
Quantity
100 μL
Datasheet
Datasheet
Reactivity
Human
Application
WB, IHC, ELISA, IF
Validations
- (4)
Cat. No.
ABIN7158768
Quantity
100 μg
Datasheet
Datasheet
Recommended GAA ELISA试剂盒
Product
Reactivity
Analytical Method
Validations
Cat. No.
Quantity
Datasheet
Reactivity
Human
Analytical Method
Quantitative Sandwich ELISA
Validations
- (5)
Cat. No.
ABIN6574146
Quantity
96 tests
Datasheet
Datasheet
Reactivity
Human
Analytical Method
Quantitative Sandwich ELISA
Validations
- (1)
Cat. No.
ABIN6967040
Quantity
96 tests
Datasheet
Datasheet
Reactivity
Mouse
Analytical Method
Quantitative Sandwich ELISA
Validations
- (1)
Cat. No.
ABIN6956130
Quantity
96 tests
Datasheet
Datasheet
Recommended GAA 蛋白
Product
Reactivity
Source
Validations
Cat. No.
Quantity
Datasheet
Reactivity
Human
Source
Wheat germ
Validations
- (1)
Cat. No.
ABIN1354615
Quantity
10 μg
Datasheet
Datasheet
Reactivity
Human
Source
Wheat germ
Validations
- (1)
Cat. No.
ABIN1354617
Quantity
10 μg
Datasheet
Datasheet
Reactivity
Rat
Source
Escherichia coli (E. coli)
Validations
Cat. No.
ABIN7404923
Quantity
100 μg
Datasheet
Datasheet
Latest Publications for our GAA Products
: "Proteasome Inhibitor Bortezomib Enhances the Activity of Multiple Mutant Forms of Lysosomal α-Glucosidase in Pompe Disease." in: JIMD reports, Vol. 18, pp. 33-9, (2015) (PubMed).: "Discovery of a novel noniminosugar acid ? glucosidase chaperone series." in: Journal of medicinal chemistry, Vol. 55, Issue 17, pp. 7546-59, (2012) (PubMed).
: "Glycosylation-related gene expression is linked to differentiation status in glioblastomas undifferentiated cells." in: Cancer letters, Vol. 312, Issue 1, pp. 24-32, (2011) (PubMed).
Synonyms and alternative names related to GAA
glucosidase alpha, acid (GAA), glucosidase, alpha, acid (Gaa), transmembrane and coiled-coil domain family 1 (TMCC1), glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II) (gaa), glucosidase alpha, acid (Gaa), E430018M07Rik, LYAGProtein level used designations for GAA
- acid maltase
- aglucosidase alfa
- lysosomal alpha-glucosidase
- acid (Pompe disease, glycogen storage disease type II)
- acid alpha-glucosidase
- glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II)
- transmembrane and coiled-coil domains protein 1
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