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Pegvaliase

Phase 3

Phenylketonuria (PKU) | Small molecule | Metabolic |BioMarin Pharmaceutical Inc.|Last Updated: Jul 21, 2026

Success Probability

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Market & Valuation

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Trial Design

RandomizedCONTROLLEDDMC
Total Trials2
Total Enrollment92

FDA Designations

No designations recorded

Clinical trial landscape

Pegvaliase · 2 trials · 1 indication

Phase 3 2
NCT05270837Study to Evaluate the Safety and Efficacy of Pegvaliase in Adolescents (Ages 12-17) With PhenylketonuriaPhenylketonuria (PKU)
ACTIVE NOT_RECRUITING55 Analytics
NCT03694353Safety and Efficacy of Self Administered Injections of Pegvaliase (>40mg/Day Dose) in Adults With PKUPhenylketonuria (PKU)
COMPLETED37 Analytics
PHASE3ACTIVE NOT_RECRUITING
Study to Evaluate the Safety and Efficacy of Pegvaliase in Adolescents (Ages 12-17) With Phenylketonuria
Phenylketonuria (PKU)Unlock trial analytics
PHASE3COMPLETED
Safety and Efficacy of Self Administered Injections of Pegvaliase (>40mg/Day Dose) in Adults With PKU
Phenylketonuria (PKU)Unlock trial analytics

Study Endpoints

Primary Endpoints

Change From Baseline in Blood Phe Concentration Following 72 Weeks (Average of the Week 69 and Week 73) on Study.
Baseline to Week 72 (average of the Week 69 and Week 73)

The blood Phe measurement following 72 weeks on study is defined as the average of the Week 69 and Week 73 blood Phe measurements. Change from baseline in blood Phe will be compared between participants in the active (pegvaliase) and the control (diet-only) treatment arms. Baseline blood Phe concentration at Part 1 was defined as the average of 3 pre-treatment measurements collected between Screening/Run-in (2) and Day 1 pre-dose (1).

Incidence of Treatment-emergent Adverse Events (Part 1)
Up to Week 73

Treatment-emergent adverse events (TEAE) is defined as any AE that newly appeared or worsened in severity after first dose of pegvaliase (pegvaliase arm) or on or after study day 1 (diet-only arm) until 30 days after last dose of the study. Serious adverse event (SAE).

Percentage of Participants With Positive Anti-pegvaliase Total Antibody (TAb)
Baseline (Day 1), Week 73

The anti-pegvaliase total antibody (TAb) method captures drug-binding antibodies comprised of different isotypes and specificities. Antibody Positivity = number of subjects testing positive at any study visit / total number of subjects at study visit. The baseline was defined as the last non-missing measurement collected prior to the first pegvaliase dose (pegvaliase arm) or prior to or on Visit 1 date (diet-only arm)

Percentage of Participants With Positive PAL IgG Antibody
Baseline (Day 1), Week 73

The presence of immunoglobulin G (IgG) Antibodies against phenylalanine ammonia lyase (PAL) is measured overtime. The baseline was defined as the last non-missing measurement collected prior to the first pegvaliase dose (pegvaliase arm) or prior to or on Visit 1 date (diet-only arm)

Percentage of Participants With Positive PAL IgM Antibody
Baseline (Day 1), Week 73

The presence of immunoglobulin M (IgM) Antibodies against PAL (phenylalanine ammonia lyase) is measured overtime. PAL IgM was positive if the titer value was greater than or equal to the median of all baseline PAL IgM titers. The baseline was defined as the last non-missing measurement collected prior to the first pegvaliase dose (pegvaliase arm) or prior to or on Visit 1 date (diet-only arm)

Percentage of Participants With Positive PEG IgG Antibody
Baseline (Day 1), Week 73

The presence of IgG Antibodies against polyethylene glycol (PEG) is measured overtime The baseline was defined as the last non-missing measurement collected prior to the first pegvaliase dose (pegvaliase arm) or prior to or on Visit 1 date (diet-only arm)

Percentage of Participants With Positive PEG IgM Antibody
Baseline (Day 1), Week 73

The presence of IgM Antibodies against PEG (polyethylene glycol) is measured overtime The baseline was defined as the last non-missing measurement collected prior to the first pegvaliase dose (pegvaliase arm) or prior to or on Visit 1 date (diet-only arm)

Percentage of Participants With Positive Neutralizing Antibodies (NAb)
Baseline (Day 1), Week 73

Neutralizing antibodies (NAbs) capable of inhibiting the enzymatic activity of pegvaliase were measured. The baseline was defined as the last non-missing measurement collected prior to the first pegvaliase dose (pegvaliase arm) or prior to or on Visit 1 date (diet-only arm)

Number of Participants With Treatment-emergent Adverse Events (TEAEs) as Assessed by CTCAE v5.0
Up to Day 741 (approximately Week 106)

A treatment-emergent AE was defined as any adverse event (AE) newly appearing or worsened in severity following initiation of study drug until 4 weeks after last dose of pegvaliase

Change in Blood Phe Concentration
The Outcome Measure Data Table below uses the 'analysis visit' as defined by the mapping rule in the SAP. The last Phe measurement was mapped to 'analysis visit' week 121. The actual date of the last Phe measurement was day 836 (approximately Week 119).

Change in blood phenylalanine (Phe) concentration from Parent Study baseline (naïve/pretreatment).

Secondary Endpoints

Change From Baseline in Total Dietary Protein Intake (i.e., Medical Food and/or Intact Food) Following 72 Weeks (Average of Week 69 and Week 73) on Study
Baseline to Week 72 (average of Week 69 and week 73)
Percentage Change From Baseline in Total Dietary Protein Intake (i.e., Medical Food and/or Intact Food) Following 72 Weeks (Average of Week 69 and Week 73) on Study
Baseline to Week 72 (average of Week 69 and week 73)
Pegvaliase Tmax (Week 73 Intensive PK)
Week 73
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Study Design & Arms

AllocationRANDOMIZED
MaskingNONE
ModelPARALLEL
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
PegvaliaseEXPERIMENTALTreatment Arm
Diet OnlyOTHERParticipants will be managing their PKU with diet alone. Participants in the diet-only control arm will be required to maintain and adjust dietary and medical protein food intake through Week 72, initiating pegvaliase treatment beginning Week 73 and, from Weeks 73 through 215.

Interventions

NameTypeDescription
PegvaliaseDRUGPegvaliase 2.5mg/10mg/20mg/40mg/60mg self-administered from 1 time up to 7 times a week
Diet OnlyOTHERDiet Control
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Eligibility Criteria

Age Range12 Years to 17 Years
SexALL
Healthy VolunteersNo
Study Sites16

Inclusion Criteria * Is 12 to 17 years old (US), inclusive, or 12 to 15 years (EU), inclusive, at the start of the Screening/Run-in Period (Day -28). * Diagnosis of PKU and failure to maintain recommended blood Phe levels on existing management (sapropterin dihydrochloride and Phe-restricted diet) ...

Countries:United StatesGermany
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Competitive Landscape -Phenylketonuria 12 trials (matched to "Phenylketonuria (PKU)")

Recent Changes (Last 90 Days)

LOWJul 21, 2026NCT05270837enrollmentType: changed

Frequently asked questions about Pegvaliase

What is Pegvaliase used for in Phenylketonuria (PKU)?

Pegvaliase is an investigational therapy being developed for the treatment of Phenylketonuria (PKU), a metabolic disorder. It is currently in Phase 3 clinical development and is not yet approved by the FDA. The drug is being studied in both adults and adolescents with PKU.

Who makes Pegvaliase?

Pegvaliase is being developed by BioMarin Pharmaceutical Inc., a biopharmaceutical company traded on the NASDAQ under the ticker symbol BMRN. BioMarin is conducting Phase 3 clinical trials to evaluate the safety and efficacy of Pegvaliase in patients with Phenylketonuria (PKU).

What phase is Pegvaliase in?

Pegvaliase is currently in Phase 3 clinical development for the treatment of Phenylketonuria (PKU). It is an investigational drug and has not received FDA approval. Two Phase 3 trials have been conducted or are ongoing, with one completed and one active but not recruiting.

What clinical trials is Pegvaliase in?

Pegvaliase has been studied in two Phase 3 clinical trials. The first, NCT03694353, evaluated self-administered injections of Pegvaliase in adults with PKU and has been completed. The second, NCT05270837, is an active trial evaluating Pegvaliase in adolescents aged 12-17 with PKU, conducted in the United States and Germany.

Is Pegvaliase FDA approved?

Pegvaliase is not FDA approved. It is an investigational drug currently in Phase 3 clinical trials for the treatment of Phenylketonuria (PKU). The drug is being studied in both adult and adolescent populations, but it has not yet received regulatory approval.