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GAA encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Additionally we are shipping GAA Kits (16) and GAA Proteins (14) and many more products for this protein.
Showing 10 out of 54 products:
Human Polyclonal GAA Primary Antibody for IHC (p), WB - ABIN656475
Aoyama, Ozer, Demirkol, Ebara, Murase, Podskarbi, Shin, Gokcay, Okubo: Molecular features of 23 patients with glycogen storage disease type III in Turkey: a novel mutation p.R1147G associated with isolated glucosidase deficiency, along with 9 AGL mutations. in Journal of human genetics 2009
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Human Monoclonal GAA Primary Antibody for ELISA, WB - ABIN560974
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 2012
Show all 2 references for ABIN560974
GAA deficiency results in reduced mTORC1 activation that is partly responsible for the skeletal muscle wasting phenotype and can be amerliorated by leucine supplementation.
Results describe the inhibitory effects of Chana series chalcone derivatives on the activities of alpha-glucosidase (show AGLU Antibodies) and DPP-4 (show DPP4 Antibodies), and on adipocyte differentiation.
Power and torque did not change with age in control animals, but declined significantly in acid 1-4 alpha-glucosidase (show AGLU Antibodies) knockout mice in three age groups.
These studies suggest that hyase enhances penetration of enzyme into the tissues including muscle during ERT (show ELF3 Antibodies) and therefore hyase pretreatment may be important in treating Pompe disease.
Compared with controls, GAA gene expression levels in coronary artery disease (CAD (show CAD Antibodies)) patients were significantly increased, suggesting that GAA may be involved in the CAD (show CAD Antibodies) development.
Study reports on the clinical, biochemical, morphological, muscle imaging, and genetic findings of six adult Pompe patients from five unrelated families with the c.-32-13T>G GAA gene mutation in homozygous state. All patients had decreased GAA activity and elevated creatine kinase levels.
glycogen (show GYS1 Antibodies) storage disease type II is caused by deficiency of GAA activity resulting from mutation of GAA gene
RT-PCR followed by DNA sequence analysis of patients with Pompe disease revealed new variant in GAA gene resulting in aberrant splicing event.
Findings indicate that GAA c.2238G > C (p.W746C) novel mutation is the most common mutation in mainland Chinese late-onset Pompe patients, as observed in Taiwanese patients expanding the genetic spectrum of the disease.
this study shows several alterations distributed along the GAA gene in a sample of Brazilian families.
The phenotype LO-GSDII with GAA mutation in the North of Italy seems not significantly different from other LO-GSDII populations in Europe or the USA.
Data shows the largest informative family with late-onset Pompe disease described in the literature showing a peculiar complex set of mutations of GAA gene that may partially elucidate the clinical heterogeneity of this family.
7 of 27 in: Gene. 2014 Mar (show IRF1 Antibodies) 1;537(1) Novel GAA sequence variant c.1211 A>G reduces enzyme activity but not protein expression in infantile and adult onset Pompe disease.
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.
acid (Pompe disease, glycogen storage disease type II)
, acid alpha-glucosidase
, acid maltase
, glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II)
, lysosomal alpha-glucosidase
, aglucosidase alfa