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Pegvaliase

Phase 3

Phenylketonuria (PKU) | Small molecule | Rare Disease |BioMarin Pharmaceutical Inc.|Last Updated: Sep 12, 2025

Success Probability
Approval Probability 71%
TA Base Rate26%
Adjusted LOA41%
ML RiskLOW_RISK
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Market & Valuation
rNPV $3.2B
Market Size $9.4B
Revenue Basis $1.6B
Competitors 6
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Trial Design
RandomizedCONTROLLEDDMCBiomarker
Total Trials2
Total Enrollment92
FDA Designations
No designations recorded
Clinical Trials (2)
NCT IDTitlePhaseStatusEnrollmentVelocityDesignStartCompletionLast UpdatedSitesCountries
NCT05270837Study to Evaluate the Safety and Efficacy of Pegvaliase in Adolescents (Ages 12-17) With PhenylketonuriaPHASE3 ACTIVE NOT_RECRUITING 55Jun 17, 2022Oct 1, 2027Sep 12, 202516 United States, Germany
NCT03694353Safety and Efficacy of Self Administered Injections of Pegvaliase (>40mg/Day Dose) in Adults With PKUPHASE3 COMPLETED 37Sep 13, 2018Jan 13, 2021Feb 17, 202217 United States
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Study Endpoints
Primary Endpoints
Change in blood Phe concentration
Treatment naïve baseline following 72 weeks on study
Incidence of treatment-emergent adverse events as assessed by CTCAE v5.0
Treatment naïve baseline following 72 weeks on study
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

Secondary Endpoints
Change in total dietary protein intake
Treatment naïve baseline following 72 weeks on study
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Study Design & Arms
AllocationRANDOMIZED
MaskingNONE
ModelPARALLEL
PurposeTREATMENT
Treatment Arms
ArmTypeDescription
PegvaliaseEXPERIMENTAL -
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 — 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
Recent Changes (Last 90 Days)
LOWMay 26, 2026NCT05270837primaryCompletionDate: changed
LOWMay 24, 2026NCT05270837studyFirstPostDate: changed