GAA
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Official Full Name
glucosidase, alpha; acid -
Overview
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 Pompes 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. -
Synonyms
GAA;glucosidase, alpha;acid;lysosomal alpha-glucosidase;glycogen storage disease type II;Pompe disease;70 kDa lysosomal alpha-glucosidase;Acid alpha glucosidase;Acid maltase;Aglucosidase alfa;Alpha glucosidase;Glucosidase alpha acid (Pompe disease glycogen storage disease type II);Glucosidase alpha acid;Glucosidase alpha;LYAG;LYAG_HUMAN;Lysosomal alpha glucosidase
Recombinant Proteins
- Human
- Mouse
- Rat
- CHO
- HEK293T
- HEK293
- E.coli
- Mammalian Cell
- Wheat Germ
- In Vitro Cell Free System
- Mamanlian cells
- Non
- His
- Myc&DDK
- GST
- His&Fc&Avi
- Flag
Involved Pathway
GAA involved in several pathways and played different roles in them. We selected most pathways GAA participated on our site, such as Galactose metabolism,Starch and sucrose metabolism,Metabolic pathways, which may be useful for your reference. Also, other proteins which involved in the same pathway with GAA were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.
| Pathway Name | Pathway Related Protein |
|---|---|
| Lysosome | ARSB,AP3M1,LIPA,ARSG,LITAF,CTSM,GBA,SGSH,LAMP3,GALCA |
| Metabolic pathways | MPST,P4HA2,ADSL,BCO1L,PMM1,PGP,NDUFS8,UQCRB,COX11,PIGS |
| Starch and sucrose metabolism | PYGB,AMY2A4,AGLA,UXS1,GANC,AMY1A,UGT1A7,AMY1B,HK2,PYGL |
| Galactose metabolism | AKR1B1,LALBA,GLA,G6PC,aLA,G6PCA.1,G6PC3,GALM,AKR1B10,GLB1 |
Protein Function
GAA has several biochemical functions, for example, . Some of the functions are cooperated with other proteins, some of the functions could acted by GAA itself. We selected most functions GAA had, and list some proteins which have the same functions with GAA. You can find most of the proteins on our site.
| Function | Related Protein |
|---|
Interacting Protein
GAA has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with GAA here. Most of them are supplied by our site. Hope this information will be useful for your research of GAA.
HIVEP1;STAT2;EP300;ypiP;q9wmx2-pro_0000037548;NUMBL;pi3p;cona_canen
Resources
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References
- Guo, MH; Hundseth, K; et al. A Distinct Triplex DNA Unwinding Activity of ChlR1 Helicase. JOURNAL OF BIOLOGICAL CHEMISTRY 290:5174-5189(2015).
- Lukas, J; Pockrandt, AM; et al. Enzyme Enhancers for the Treatment of Fabry and Pompe Disease. MOLECULAR THERAPY 23:456-464(2015).
