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alglucosidase alfa

Phase 3

Pompe Disease (Late-onset) | Monoclonal antibody | Rare Disease |Sanofi|Last Updated: Apr 28, 2015

Target and mechanism

ModalityMonoclonal antibody

Also known as Alglucosidase alfa (GZ419829)

Success Probability

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Market & Valuation

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Trial Design

RandomizedDouble-BlindPLACEBO_CONTROLLEDDMC
Total Trials1
Total Enrollment90

FDA Designations

No designations recorded

Clinical trial landscape

alglucosidase alfa · 2 trials · 6 indications

Phase 3 1Phase 2 1
NCT00158600A Placebo-Controlled Study of Safety and Effectiveness of Myozyme (Alglucosidase Alfa) in Patients With Late-Onset Pompe DiseasePompe Disease (Late-onset)
COMPLETED90 Analytics
PHASE3COMPLETED
A Placebo-Controlled Study of Safety and Effectiveness of Myozyme (Alglucosidase Alfa) in Patients With Late-Onset Pompe Disease
Pompe Disease (Late-onset)Unlock trial analytics

Study Endpoints

Primary Endpoints

Summary of Patients Reporting Treatment-Emergent Adverse Events
weeks 0-78

Overall safety summary of patients experiencing Adverse Events (AEs), Serious Adverse Events (SAEs), treatment-related AEs, and Infusion Associated Reactions (IARs). Summary is based on Treatment-emergent AEs (TEAEs), defined as AEs that occurred following the initiation of study treatment, i.e., alglucosidase alfa or placebo.

Mean Distance Walked as Measured by Six-minute Walk Test (6MWT) at Weeks 0 and 78, and Mean Change From Baseline
weeks 0, 78

Mean distance walked gives an indication of functional endurance. The greater the distance, the greater the endurance. Mean values of distance walked in a six-minute walk test are offered for baseline, week 78 (or last available observation), and the mean change from baseline (at week 78 or last available post-baseline observation).

Percent of Predicted Forced Vital Capacity (FVC)
weeks 0, 78

Forced vital capacity is a standard pulmonary function test used to quantify respiratory muscle weakness. Forced vital capacity (FVC) is the volume of air that can forcibly be blown out after full inspiration in the upright position, measured in liters. Predicted forced vital capacity is based on a formula using sex, age and height of a person, and is an estimate of healthy lung capacity. Percent of predicted FVC = (observed value)/(predicted value) \* 100%.

Recombinant Human Acid Alpha-Glucosidase (rhGAA) Pharmacokinetic Parameters: Area Under the Curve (AUC)
weeks 0, 12 and 52

Area under the plasma concentration versus time curve from time zero (pre-dose) to 16 hours after the end of infusion. Blood sample time points were 0 (before the start of the infusion), 1 and 2 hours after the start of infusion, end of the infusion, and then 0.25, 0.5, 1, 2, 3, 4, 8, 12,and 16 hours after the end of the infusion (with a 5-minute window for time-points after the start of infusion). Pooled figures combine the values for the three timeframes.

Recombinant Human Acid Alpha-Glucosidase (rhGAA) Pharmacokinetic Parameters: Mean Maximum Plasma Concentration(Cmax)
weeks 0, 12, 52

Maximum plasma concentration observed in blood samples taken at the following time points: 0 (before the start of the infusion), 1 and 2 hours after the start of infusion, end of the infusion, and then 0.25, 0.5, 1, 2, 3, 4, 8, 12,and 16 hours after the end of the infusion (with a 5-minute window for time-points after the start of infusion). Pooled figures combine the values for the three timeframes.

Recombinant Human Acid Alpha-Glucosidase (rhGAA) Pharmacokinetic Parameters: Mean Time to Maximum Plasma Concentration(Tmax)
weeks 0, 12, 52

Time to maximum plasma concentration observed in blood samples taken at the following time points: 0 (before the start of the infusion), 1 and 2 hours after the start of infusion, end of the infusion, and then 0.25, 0.5, 1, 2, 3, 4, 8, 12,and 16 hours after the end of the infusion (with a 5-minute window for time-points after the start of infusion). Pooled figures combine the values for the three timeframes.

Evaluate safety, pharmacokinetics and pharmacodynamics
52 weeks
Evaluate differences in skeletal muscle gene expression in sibling pair with identical GAA mutations
52 weeks
Evaluate differences in skeletal muscle expression prior to and after ERT
52 weeks

Secondary Endpoints

Percent Predicted Proximal Muscle Strength of the Lower Limbs as Measured by Quantitative Muscle Testing (QMT)
weeks 0, 78
Health-related Quality of Life Survey Values Related to Physical Components as Measured by the Medical Outcomes Study (MOS) Short Form-36 Health Survey
weeks 0, 78
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Study Design & Arms

AllocationRANDOMIZED
MaskingQUADRUPLE
ModelPARALLEL
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
alglucosidase alfaACTIVE_COMPARATORIntravenous (IV) infusions of alglucosidase alfa at 20 milligrams (mg)/kilogram (kg) of body weight every other week (qow) for 78 weeks.
PlaceboPLACEBO_COMPARATORIntravenous (IV) infusions of placebo every other week (qow) for 78 weeks.
1EXPERIMENTAL -

Interventions

NameTypeDescription
alglucosidase alfaBIOLOGICALIV infusion of 20mg/kg; qow for 78 weeks.
PlaceboDRUGPlacebo Comparator; qow for 78 weeks.
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Eligibility Criteria

Age Range8 Years to N/A
SexALL
Healthy VolunteersNo
Study Sites8

Inclusion Criteria: * Patient must provide signed, informed consent prior to performing any study-related procedures. * Patient must have a diagnosis of Pompe disease based on deficient endogenous GAA activity in cultured skin fibroblasts of less than or equal to 40% of the normal mean of the testi...

Countries:United StatesFranceNetherlands
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Frequently asked questions about alglucosidase alfa

What is Alglucosidase alfa used for?

Alglucosidase alfa is used for Glycogen Storage Disease Type II, also known as Pompe Disease, specifically in the late-onset form. It is an investigational therapy being developed by Sanofi for this rare disease.

What does Alglucosidase alfa target?

Alglucosidase alfa targets the underlying enzyme deficiency in Pompe Disease. It is a form of recombinant human acid alpha-glucosidase (rhGAA) designed to replace the missing enzyme that leads to glycogen accumulation in cells.

Who makes Alglucosidase alfa?

Sanofi (ticker: SNY) is developing Alglucosidase alfa. The drug is being studied for the treatment of Glycogen Storage Disease Type II and late-onset Pompe Disease.

What phase is Alglucosidase alfa in?

Alglucosidase alfa is in Phase 2 clinical development. It is an investigational drug and has not been approved by regulatory authorities. One Phase 2 trial has been completed, along with a Phase 3 trial.

What clinical trials is Alglucosidase alfa in?

Alglucosidase alfa has been studied in two completed trials. NCT00051935 was a Phase 2 study in siblings with Glycogen Storage Disease Type II, and NCT00158600 was a Phase 3 placebo-controlled study in patients with late-onset Pompe Disease.

Is Alglucosidase alfa the same as GZ419829?

Yes, Alglucosidase alfa is also known as GZ419829. This alternative name may be used in some clinical or research contexts to refer to the same drug.