Catalog No.
DHC80031
Expression system
Mammalian Cells
Species reactivity
Human
Isotype
A recombinant GAA/Aglucosidase alfa
Clonality
Monoclonal
Target
GAA, Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa
Concentration
1 mg/ml
Endotoxin level
Please contact with the lab for this information.
Purity
>95% as determined by SDS-PAGE.
Purification
Purified by Nickel column.
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.
Clone ID
Aglucosidase Alfa
Improving the treatment of Pompe disease with enzyme replacement therapy: current strategies and clinical evidence., PMID:40237692
Biochemical and Genetic Testing of GAA in Over 30.000 Symptomatic Patients Suspected to Be Affected With Pompe Disease., PMID:40225932
A stable GH31 α-glucosidase as a model system for the study of mutations leading to human glycogen storage disease type II., PMID:39995088
Clinical and genetic spectrums of Pompe disease in Duhok city, Kurdistan region, Iraq., PMID:39910942
Rescue of common and rare exon 2 skipping variants of the GAA gene using modified U1 snRNA., PMID:39905333
Engineering of a lysosomal-targeted GAA enzyme., PMID:39846949
A Real-World Data Analysis of Alglucosidase Alfa in the FDA Adverse Event Reporting System (FAERS) Database., PMID:39833603
Challenges in multinational rare disease clinical studies during COVID-19: regulatory assessment of cipaglucosidase alfa plus miglustat in adults with late-onset Pompe disease., PMID:39775064
Predicting the phenotype of Pompe Disease from features of GAA variants., PMID:39775060
Quantitative Systems Pharmacology-Based Digital Twins Approach Supplements Clinical Trial Data for Enzyme Replacement Therapies in Pompe Disease., PMID:39632463
[Importance of early treatment and quantitative evaluation of enzyme replacement therapy for Pompe disease: alglucosidase alfa post-marketing surveillance additional analysis]., PMID:39566964
Home infusion experience in patients with Pompe disease receiving avalglucosidase alfa during three clinical trials., PMID:39566417
Switching treatment to cipaglucosidase alfa plus miglustat positively affects patient-reported outcome measures in patients with late-onset Pompe disease., PMID:39535661
Pompe disease: Unmet needs and emerging therapies., PMID:39418752
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
Mutation Spectrum of GAA Gene in Pompe Disease: Current Knowledge and Results of an Italian Study., PMID:39273088
High-risk screening for late-onset Pompe disease in China: An expanded multicenter study., PMID:39227307
Clinical features and genetic analysis of 5 cases of infantile-type glycogen storage disease type II: Case reports., PMID:39213226
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
Advances in Pompe Disease Treatment: From Enzyme Replacement to Gene Therapy., PMID:39134822
Enzyme replacement therapy and immunotherapy lead to significant functional improvement in two children with Pompe disease: a case report., PMID:39020349
Real-world data of in-hospital administration of alglucosidase alfa in French patients with Pompe disease: results from the National Claims Database., PMID:38963441
Mutational spectrum and genotype-phenotype correlation in Mexican patients with infantile-onset and late-onset Pompe disease., PMID:38958145
Changes in forced vital capacity over ≤ 13 years among patients with late-onset Pompe disease treated with alglucosidase alfa: new modeling of real-world data from the Pompe Registry., PMID:38896264
Regulatory news: Cipaglucosidase alfa-atga (Pombiliti) coadministered with Miglustat (Opfolda) for adults with late-onset Pompe disease., PMID:38768612
Optimizing treatment outcomes: immune tolerance induction in Pompe disease patients undergoing enzyme replacement therapy., PMID:38715621
Acid α-glucosidase (GAA) activity and glycogen content in muscle biopsy specimens of patients with Pompe disease: A systematic review., PMID:38698877
Human fecal alpha-glucosidase activity and its relationship with gut microbiota profiles and early stages of intestinal mucosa damage., PMID:38614290
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
104-week efficacy and safety of cipaglucosidase alfa plus miglustat in adults with late-onset Pompe disease: a phase III open-label extension study (ATB200-07)., PMID:38418563
Effect of intracerebroventricular administration of alglucosidase alfa in two mouse models of Lafora disease: Relevance for clinical practice., PMID:38341935
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
Higher dose alglucosidase alfa is associated with improved overall survival in infantile-onset Pompe disease (IOPD): data from the Pompe Registry., PMID:38057861
Are Anti-rhGAA Antibodies a Determinant of Treatment Outcome in Adults with Late-Onset Pompe Disease? A Systematic Review., PMID:37759814
Pharmacokinetics of Alglucosidase Alfa Manufactured at the 4000-L Scale in Participants with Pompe Disease: A Phase 3/4 Open-Label Study., PMID:37705424
Increasing Enzyme Mannose-6-Phosphate Levels but Not Miglustat Coadministration Enhances the Efficacy of Enzyme Replacement Therapy in Pompe Mice., PMID:37679046
Genotype, phenotype and treatment outcomes of 17 Malaysian patients with infantile-onset Pompe disease and the identification of 3 novel GAA variants., PMID:37542277
Glycoproteomics of a Single Protein: Revealing Tens of Thousands of Myozyme Glycoforms by Hybrid HPLC-MS Approaches., PMID:37478974
Natural History and Phenotypic Spectrum of GAA-FGF14 Sporadic Late-Onset Cerebellar Ataxia (SCA27B)., PMID:37470282
Home-Based Infusion of Alglucosidase Alfa Can Safely be Implemented in Adults with Late-Onset Pompe Disease: Lessons Learned from 18,380 Infusions., PMID:37326923
Model-Informed Approach Supporting Approval of Nexviazyme (Avalglucosidase Alfa-ngpt) in Pediatric Patients with Late-Onset Pompe Disease., PMID:36653728
Long-Term Experience with Anaphylaxis and Desensitization to Alglucosidase Alfa in Pompe Disease., PMID:36623499
Greater Efficacy of Avalglucosidase vs Alglucosidase Alfa in Adult Pompe Disease? The Jury Is Still Out., PMID:35618439
5-C-Branched Deoxynojirimycin: Strategy for Designing a 1-Deoxynojirimycin-Based Pharmacological Chaperone with a Nanomolar Affinity for Pompe Disease., PMID:35072486
Late Onset Pompe Disease with Novel Mutations and Atypical Phenotypes., PMID:34864681
Effect of alglucosidase alfa dosage on survival and walking ability in patients with classic infantile Pompe disease: a multicentre observational cohort study from the European Pompe Consortium., PMID:34822769
For research use only. Not for human or drug use.
