Catalog No.
DHC80030
Expression system
Mammalian Cells
Species reactivity
Human
Clonality
Monoclonal
Target
GAA, Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa
Concentration
0.96 mg/ml
Endotoxin level
Please contact with the lab for this information.
Purity
>95% as determined by SDS-PAGE.
Purification
Protein A/G purified from cell culture supernatant.
Accession
P10253
Applications
Research Grade Biosimilar
Form
Liquid
Storage buffer
0.01M PBS, pH 7.4.
Stability and Storage
Use a manual defrost freezer and avoid repeated freeze-thaw cycles. Store at 4°C short term (1-2 weeks). Store at -20°C 12 months. Store at -80°C long term.
Alternative Names
GZ-402666, avalglucosidase alfa-ngpt
Clone ID
Nexviazyme
Enzyme replacement therapies in adults with Pompe disease: from trials to real-world data., PMID:40471681
The Mini-COMET Clinical Trial: Safety and Efficacy of Avalglucosidase Alfa after 97 Weeks of Treatment in Children with Infantile-Onset Pompe Disease Previously Treated with Alglucosidase Alfa., PMID:40449831
Defining clinically meaningful thresholds for forced vital capacity in patients with neuromuscular disorders: Lessons learned from the COMET study in Pompe disease., PMID:40397025
Improving the treatment of Pompe disease with enzyme replacement therapy: current strategies and clinical evidence., PMID:40237692
Successful desensitization protocol to alglucosidase and avalglucosidase alfa in a patient with infantile-onset Pompe disease., PMID:40206190
Efficacy of transitioning from alglucosidase alfa to avalglucosidase alfa in infantile-onset Pompe disease: A single-center cohort analysis., PMID:39927452
Management of presymptomatic juvenile patients with late-onset Pompe disease (LOPD)., PMID:39879733
Efficacy and safety of avalglucosidase alfa in Japanese patients with late-onset and infantile-onset Pompe diseases: A case series from clinical trials., PMID:39835171
Corrigendum to "Home infusion experience in patients with Pompe disease receiving avalglucosidase alfa during three clinical trials" [Molecular Genetics and Metabolism, 143 (2024) 108608]., PMID:39787886
Advances in Disease-Modifying Therapeutics for Chronic Neuromuscular Disorders., PMID:39708835
Clinical modeling of motor function to predict treatment efficacy and enable in silico treatment comparisons in infantile-onset Pompe disease., PMID:39670959
Quantitative Systems Pharmacology-Based Digital Twins Approach Supplements Clinical Trial Data for Enzyme Replacement Therapies in Pompe Disease., PMID:39632463
Home infusion experience in patients with Pompe disease receiving avalglucosidase alfa during three clinical trials., PMID:39566417
Pompe disease: Unmet needs and emerging therapies., PMID:39418752
Optimizing clinical outcomes: The journey of twins with CRIM-negative infantile-onset Pompe disease on high-dose enzyme replacement therapy and immunomodulation., PMID:39314994
Comparing the efficacy of cipaglucosidase alfa plus miglustat with other enzyme replacement therapies for late-onset Pompe disease: a network meta-analysis utilizing patient-level and aggregate data., PMID:39287071
Avalglucosidase alfa in infantile-onset Pompe disease: A snapshot of real-world experience in Italy., PMID:39161458
Clinical insight meets scientific innovation to develop a next generation ERT for Pompe disease., PMID:39154400
Long-term observation of patients with advanced late-onset Pompe disease undergoing enzyme replacement therapy: A 15-year observation in a single center., PMID:39142946
Efficacy of avalglucosidase alfa on forced vital capacity percent predicted in treatment-naïve patients with late-onset Pompe disease: A pooled analysis of clinical trials., PMID:39035044
Real-life effectiveness 1 year after switching to avalglucosidase alfa in late-onset Pompe disease patients worsening on alglucosidase alfa therapy: A French cohort study., PMID:38587143
Real-world evidence study finds no new-onset diabetes or drug-related hyperglycemia in Pompe disease patients treated with avalglucosidase alfa., PMID:38469102
Real-world outcomes from a series of patients with late onset Pompe disease who switched from alglucosidase alfa to avalglucosidase alfa., PMID:38313679
Evaluating avalglucosidase alfa for the management of late-onset Pompe disease., PMID:38261315
Applying the win ratio method in clinical trials of orphan drugs: an analysis of data from the COMET trial of avalglucosidase alfa in patients with late-onset Pompe disease., PMID:38216959
Effect of avalglucosidase alfa on disease-specific and general patient-reported outcomes in treatment-naïve adults with late-onset Pompe disease compared with alglucosidase alfa: Meaningful change analyses from the Phase 3 COMET trial., PMID:38184428
Post-hoc Nonparametric Analysis of Forced Vital Capacity in the COMET Trial Demonstrates Superiority of Avalglucosidase Alfa vs Alglucosidase Alfa., PMID:38160363
Enzyme replacement therapy for late-onset Pompe disease., PMID:38084761
Increasing Enzyme Mannose-6-Phosphate Levels but Not Miglustat Coadministration Enhances the Efficacy of Enzyme Replacement Therapy in Pompe Mice., PMID:37679046
Measurement Properties of 2 Novel PROs, the Pompe Disease Symptom Scale and Pompe Disease Impact Scale, in the COMET Study., PMID:37559825
Population Pharmacokinetic Modeling and Determination of Individual Exposure to Avalglucosidase Alfa in Adolescent and Adult Patients With Late-Onset Pompe Disease: Analysis of Pooled Data From Phase I to III Clinical Trials., PMID:37556417
Population pharmacokinetic modeling and dosing simulation of avalglucosidase alfa for selecting alternative dosing regimen in pediatric patients with late-onset pompe disease., PMID:37535240
[Drugs for Genetic Myopathy]., PMID:37194523
Efficacy and Safety of Avalglucosidase Alfa in Patients With Late-Onset Pompe Disease After 97 Weeks: A Phase 3 Randomized Clinical Trial., PMID:37036722
Transcriptomic characterization of clinical skeletal muscle biopsy from late-onset Pompe patients., PMID:36774918
Model-Informed Approach Supporting Approval of Nexviazyme (Avalglucosidase Alfa-ngpt) in Pediatric Patients with Late-Onset Pompe Disease., PMID:36653728
The new horizons for treatment of Late-Onset Pompe Disease (LOPD)., PMID:36609019
Safety and efficacy of avalglucosidase alfa in individuals with infantile-onset Pompe disease enrolled in the phase 2, open-label Mini-COMET study: The 6-month primary analysis report., PMID:36542086
Therapeutic thoroughfares for adults living with Pompe disease., PMID:35942661
Regulatory news: Avalglucosidase alfa-ngpt (Nexviazyme) for late-onset Pompe disease-FDA approval summary., PMID:35929375
Long-term Safety and Efficacy of Avalglucosidase Alfa in Patients With Late-Onset Pompe Disease., PMID:35618441
Greater Efficacy of Avalglucosidase vs Alglucosidase Alfa in Adult Pompe Disease? The Jury Is Still Out., PMID:35618439
Safety and efficacy of avalglucosidase alfa versus alglucosidase alfa in patients with late-onset Pompe disease (COMET): a phase 3, randomised, multicentre trial., PMID:34800399
Avalglucosidase alfa: First Approval., PMID:34591286
Avalglucosidase Alfa-ngpt., PMID:34587240
Safety, tolerability, pharmacokinetics, pharmacodynamics, and exploratory efficacy of the novel enzyme replacement therapy avalglucosidase alfa (neoGAA) in treatment-naïve and alglucosidase alfa-treated patients with late-onset Pompe disease: A phase 1, open-label, multicenter, multinational, ascending dose study., PMID:30770310
For research use only. Not for human or drug use.
