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GLA encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. Additionally we are shipping GLA Kits (34) and GLA Proteins (17) and many more products for this protein.
Showing 10 out of 104 products:
Human Polyclonal GLA Primary Antibody for EIA, WB - ABIN453055
Ioannou, Zeidner, Grace, Desnick: Human alpha-galactosidase A: glycosylation site 3 is essential for enzyme solubility. in The Biochemical journal 1998
Show all 2 references for 453055
Human Polyclonal GLA Primary Antibody for IHC, IHC (p) - ABIN4280010
Xu, Lun, Brignol, Hamler, Schilling, Frascella, Sullivan, Boyd, Chang, Soska, Garcia, Feng, Yasukawa, Shardlow, Churchill, Ketkar, Robertson, Miyamoto, Mihara, Benjamin, Lockhart, Hirato, Fowles et al.: Coformulation of a Novel Human α-Galactosidase A With the Pharmacological Chaperone AT1001 Leads to Improved Substrate Reduction in Fabry Mice. ... in Molecular therapy : the journal of the American Society of Gene Therapy 2015
Cow (Bovine) Polyclonal GLA Primary Antibody for WB - ABIN2785641
Auray-Blais, Cyr, Ntwari, West, Cox-Brinkman, Bichet, Germain, Laframboise, Melançon, Stockley, Clarke, Drouin: Urinary globotriaosylceramide excretion correlates with the genotype in children and adults with Fabry disease. in Molecular genetics and metabolism 2008
we review the various types of GLA (show NAT8 Antibodies) variants and recommend that pathogenicity be considered only when associated with elevated globotriaosylceramide in disease-relevant organs and tissues as analyzed by mass spectrometry.
findings revealed the alternative splicing mechanism of GLA (show NAT8 Antibodies) (IVS4+919G>A), and a potential treatment for this specific genetic type of Fabry disease by amiloride in the future
Results found a novel heterozygous stop codon mutation in exon 1 of the GLA (show NAT8 Antibodies) gene in female patients with Fabry Disease with methylation in the non-mutated allele thought to be associated with the clinical severity of the disease.
Study described the demographic data, wide clinical spectrum of phenotypes, and GLA (show NAT8 Antibodies) mutation spectrum of Fabry disease in Korea. Most of the patients had classical Fabry disease, with a 4 times higher incidence than that of late-onset Fabry disease, indicating an underdiagnosis of mild, late-onset Fabry disease.
we reviewed other small molecules that were reported to have a stabilizing effect on some GLA (show NAT8 Antibodies) missense mutations in vitro and might be developed to act in synergy or as an alternative to 1-deoxygalactonojirimycin
No pathogenic mutations in the coding regions of the GLA (show NAT8 Antibodies) gene were identified in this group of patients and thus no Fabry disease was found in this study.
High desphospho-uncarboxylated matrix Gla protein (show MGP Antibodies) level, reflecting a poor vitamin K status, seems to be associated with kidney damage and may be also a marker of cardiovascular risk in CKD patients
Case Report: Kidney transplantation from a mother with unrecognized Fabry disease to her son with low alpha-galactosidase A activity.
p.M187R GLA (show NAT8 Antibodies) mutation in Fabry disease causes a severe systemic and ophthalmologic phenotype, in both male and female patients.
The results of the current study suggest that the GLA (show NAT8 Antibodies) haplotype D313Y does not lead to severe organ manifestations as seen in genotypes known to be causal for classical Fabry disease.
Mice with alpha-galactosidase A deficiency show age-dependent and distinct deficits of the sensory system.
In oocyte meiosis, GM130 (show GOLGA2 Antibodies) localization and expression patterns are regulated by FMNL1 (show FMNL1 Antibodies).
The histological changes in Gla KO mice better resemble the type 2 later-onset phenotype observed in patients with residual alpha-galactosidase A activity.
our findings imply that the alpha-GalA KO mouse is a good model in which to study the peripheral small fiber neuropathy exhibited by FD patients
we demonstrate an age-dependent microvasculopathy of the mesenteric artery in a murine model of Fabry disease (galactosidase A-knockout mice) resulting from dysregulation of the vascular homeostatic enzyme endothelial nitric oxide synthase (eNOS (show NOS3 Antibodies))
GM130 (show GOLGA2 Antibodies) regulates microtubule organization and might cooperate with the MAPK (show MAPK1 Antibodies) pathway to play roles in spindle organization, migration and asymmetric division during mouse oocyte maturation
It suggested that there could be a combination of GLA deficiency and FVL (show F5 Antibodies) or other thrombosis-related gene defect in patients with genetic severe early-onset thrombosis.
present Toll (show TLR4 Antibodies)-like receptor-dependent negative regulation of alpha-Gal-A as a mechanistic link between pathogen recognition and self lipid antigen induction for natural killer T cells
Developed a novel recombinant lentiviral vector that engineers expression of alpha-galactosidase. Analysis of tissues at 26 wks demonstrated similar alpha-gal A enzyme activities but enhanced Gb3 reduction in hearts and kidneys compared with control.
alpha-galactosidase A deficiency could be an important genetic modifier for the enhanced thrombosis associated with FV Leiden associated thrombosis.
This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties.
, galactosidase alpha
, Alpha-galactosidase A
, alpha-D-galactosidase A
, alpha-galactosidase A-like
, alpha-galactosidase A
, agalsidase alfa
, alpha-D-galactoside galactohydrolase 1
, alpha-gal A
, alpha-D-galactoside galactohydrolase
, 130 kDa cis-Golgi matrix protein
, Golgin subfamily A member 2
, SY11 protein