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GAA Products

(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 Antibodies

High quality antibodies with extensive validation data.

GAA ELISA Kits

Reliable ELISA kits for a wide range of species.

GAA Proteins

Proteins for various applications incl. WB, ELISA, IF etc.

Recommended GAA Antibodies

Product
Reactivity
Application
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Application WB, IHC, IHC (p)
Validations
  • (5)
Cat. No. ABIN656475
Quantity 400 μL
Datasheet 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

Recommended GAA ELISA Kits

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 Proteins

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

Shimada, Nishimura, Hoshina, Kobayashi, Higuchi, Eto, Ida, Ohashi: "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).

Xiao, Westbroek, Motabar, Lea, Hu, Velayati, Zheng, Southall, Gustafson, Goldin, Sidransky, Liu, Simeonov, Tamargo, Ribes, Matalonga, Ferrer, Marugan: "Discovery of a novel noniminosugar acid ? glucosidase chaperone series." in: Journal of medicinal chemistry, Vol. 55, Issue 17, pp. 7546-59, (2012) (PubMed).

Cheray, Petit, Forestier, Karayan-Tapon, Maftah, Jauberteau, Battu, Gallet, Lalloué: "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, LYAG

Protein 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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