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GLA encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. 再加上，我们可以发GLA 试剂盒 (38) 和 GLA 蛋白 (18)和数多这个蛋白质的别的产品。
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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
Results showed that most Fabry disease patients carrying GLA (显示 NAT8 抗体) IVS4+919A did not show abnormal cardiac phenotypes. The near-absence of GLA (显示 NAT8 抗体) IVS4+919A in heart disease cohort suggested that this variant is not a frequent cause of overt heart diseases in Taiwan.
Enzyme activities (acid alpha-glucosidase (GAA), galactocerebrosidase (GALC (显示 GALC 抗体)), glucocerebrosidase (GBA (显示 GBA 抗体)), alpha-galactosidase A (GLA), alpha-iduronidase (IDUA (显示 IDUA 抗体)) and sphingomyeline phosphodiesterase-1 (SMPD-1 (显示 SMPD1 抗体))) were measured on ~43,000 de-identified dried blood spot (DBS (显示 MCF2L 抗体)) punches, and screen positive samples were submitted for DNA sequencing to obtain genotype confirmation of disease risk
This longitudinal Fabry Registry study analyzed data from patients with Fabry disease to determine the incidence and type of severe clinical events following initiation of enzyme replacement therapy (ERT (显示 ELF3 抗体)) with agalsidase beta, as well as risk factors associated with occurrence of these events
alpha-galactosidase A mutation, IVS4-type Fabry disease has features similar to those of classic Fabry disease and a higher frequency of deep white matter hyperintensities and a higher incidence of infarctions and pulvinar signs than in healthy controls
We demonstrate that the wild-type sequence harbors an hnRNP A1 (显示 HNRNPA1 抗体) and hnRNP A2/B1 (显示 HNRNPA2B1 抗体)-binding exonic splicing silencer (ESS) overlapping the 5'splice site (5'ss) that prevents pseudoexon inclusion.we demonstrate that splice switching oligonucleotide (SSO) mediated blocking of the pseudoexon 3'ss and 5'ss effectively restores normal GLA (显示 NAT8 抗体) splicing
Four patients had non-amenable mutant forms of a-Gal (显示 GAL 抗体) based on the validated cell-based assay conducted after treatment initiation and were excluded from primary efficacy analyses only.
Mesenchymal stem cells with reduced GLA (显示 NAT8 抗体) activity are prone to apoptosis and senescence due to impaired autophagy and DNA repair capacity.
we review the various types of GLA (显示 NAT8 抗体) 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 (显示 NAT8 抗体) (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 (显示 NAT8 抗体) 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.
Therefore, we confirmed the associations among Golgi function, fibrosis, and autophagy. Moreover, GOLGA2 (显示 GOLGA2 抗体) knockout mice may be a potentially valuable animal model for studying autophagy-induced fibrosis.
the GM130 (显示 GOLGA2 抗体)-deficient mouse provides a valuable model for investigating the etiology of human globozoospermia.
Mice with alpha-galactosidase A deficiency show age-dependent and distinct deficits of the sensory system.
In oocyte meiosis, GM130 (显示 GOLGA2 抗体) localization and expression patterns are regulated by FMNL1 (显示 FMNL1 抗体).
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 (显示 NOS3 抗体))
GM130 (显示 GOLGA2 抗体) regulates microtubule organization and might cooperate with the MAPK (显示 MAPK1 抗体) 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 (显示 F5 抗体) or other thrombosis-related gene defect in patients with genetic severe early-onset thrombosis.
present Toll (显示 TLR4 抗体)-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
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.
, alpha-D-galactosidase A
, alpha-D-galactoside galactohydrolase 1
, alpha-gal A
, alpha-galactosidase A
, alpha-D-galactoside galactohydrolase
, Alpha-galactosidase A
, galactosidase, alpha
, alpha-galactosidase A-like
, 130 kDa cis-Golgi matrix protein
, Golgin subfamily A member 2
, SY11 protein