Phenylketonuria

Last reviewed

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ORPHA:716OMIM:261600E70.0E70.1
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6FDA treatments20Active trials32Specialists8Treatment centers10Financial resources

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Phenylketonuria is treated with 8 medications in our database, including Sapropterin Dihydrochloride, SAPROPTERIN DIHYDROCHLORIDE, Sapropterin dihydrochloride, PCXX VANILLA ORANGE ONE MINUTE FM, Palynziq, and 3 more. 7 of these have manufacturer assistance programs available to help reduce out-of-pocket costs. Medications are manufactured by BioMarin, Pascal, Sentynl. Patients and caregivers can find copay cards, patient assistance programs, and travel grants for Phenylketonuria treatment below.

Also known as:

Clinical phenotype terms— hover any for plain English:

PhenylalaninuriaHP:0032351HyperphenylalaninemiaHP:0004923Musty odorHP:0410021
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

10 events
Feb 2026PALYNZIQ: New indication approved
FDAcompleted
Nov 2025PALYNZIQ: New indication approved
FDAcompleted
Oct 2025A Study to Evaluate the Safety and Efficacy of JNT-517 in Participants With Phenylketonuria (PKU)

Otsuka Pharmaceutical Development & Commercialization, Inc. — PHASE3

TrialRECRUITING
Sep 2025Evaluation of BH4 Responsiveness in Our PKU Patients

Sohag University — PHASE1

TrialACTIVE NOT RECRUITING
May 2025GMP Powdered Substitutes in PKU and TYR

Nutricia UK Ltd — NA

TrialRECRUITING
Apr 2025A Self-test Home-use Blood Phenylalanine Monitoring System Under the Brand Name Egoo Phe System Has Been Developed for Measurement of Phenylalanine (Phe) in Individuals Diagnosed With Phenylketonuria (PKU): The Study Purpose is to Evaluate Accuracy and Usability

Egoo Health Aps — NA

TrialENROLLING BY INVITATION
Feb 2025Effect of L-carnitine Supplementation on Phenylalanine and Brain-derived Neurotrophic Factor Levels in Infants and Children With Phenylketonuria

Mansoura University — PHASE4

TrialACTIVE NOT RECRUITING
Jan 2025Brain Aging in Phenylketonuria

Insel Gruppe AG, University Hospital Bern — NA

TrialRECRUITING
Jan 2025AAV Gene Therapy Clinical Study in Adult Classic PKU (PHEdom)

NGGT INC. — PHASE1

TrialRECRUITING
Oct 2024Development of Novel Psychological Assessment Tools and Anxiety Intervention for Phenylketonuria

University of Missouri-Columbia — NA

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

6 available

Sephience

SEPIAPTERIN· PTC Therapeutics Inc.Orphan Drug
1 INDICATIONS AND USAGE SEPHIENCE is indicated for the treatment of hyperphenylalaninemia (HPA) in adult and pediatric patients 1 month of age and older with sepiapterin-responsive phenylketonuria (PK

1 INDICATIONS AND USAGE SEPHIENCE is indicated for the treatment of hyperphenylalaninemia (HPA) in adult and pediatric patients 1 month of age and older with sepiapterin-responsive phenylketonuria (PKU). SEPHIENCE is to be used in conjunction with a phenylalanine (Phe)-restricted diet. SEPHIENCE is a phenylalanine hydroxylase (PAH) activator indicated for the treatment of hyperphenylalaninemia (HPA) in adult and pediatric patients 1 month of age and older with sepiapterin-responsive phenylketonuria (PKU). SEPHIENCE is to be used in conjunction with a phenylalanine (Phe)- restricted diet. ( 1 )

Palynziq

PEGVALIASE-PQPZ· BioMarin Pharmaceutical Inc.■ Boxed WarningOrphan Drug
1 INDICATIONS AND USAGE PALYNZIQ is indicated to reduce blood phenylalanine concentrations in adult and pediatric patients 12 years of age and older with phenylketonuria (PKU) who have uncontrolled bl

1 INDICATIONS AND USAGE PALYNZIQ is indicated to reduce blood phenylalanine concentrations in adult and pediatric patients 12 years of age and older with phenylketonuria (PKU) who have uncontrolled blood phenylalanine concentrations greater than 600 micromol/L on existing management. PALYNZIQ is a phenylalanine (Phe)-metabolizing enzyme indicated to reduce blood phenylalanine concentrations in adult and pediatric patients 12 years of age and older with phenylketonuria (PKU) who have uncontrolled blood phenylalanine concentrations greater than 600 micromol/L on existing management. ( 1 )

Sapropterin Dihydrochloride

SAPROPTERIN DIHYDROCHLORIDE· Aurobindo Pharma LimitedOrphan Drug
1 INDICATIONS AND USAGE Sapropterin dihydrochloride tablets are indicated to reduce blood phenylalanine (Phe) levels in adult and pediatric patients one month of age and older with hyperphenylalaninem

1 INDICATIONS AND USAGE Sapropterin dihydrochloride tablets are indicated to reduce blood phenylalanine (Phe) levels in adult and pediatric patients one month of age and older with hyperphenylalaninemia (HPA) due to tetrahydrobiopterin- (BH4-) responsive Phenylketonuria (PKU). Sapropterin dihydrochloride tablets are to be used in conjunction with a Phe-restricted diet. Sapropterin dihydrochloride tablets are a phenylalanine hydroxylase activator indicated to reduce blood phenylalanine (Phe) levels in adult and pediatric patients one month of age and older with hyperphenylalaninemia (HPA) due to tetrahydrobiopterin- (BH4-) responsive Phenylketonuria (PKU). Sapropterin dihydrochloride tablets are to be used in conjunction with a Phe-restricted diet. ( 1 )

Kuvan

sapropterin· BioMarin Pharmaceutical, Inc.
KUVAN is indicated to reduce blood phenylalanine (Phe) levels in adult and pediatric patients one month of age and older with hyperphenylalaninemia (HPA) due to tetrahydrobiopterin- (BH4-) responsive

KUVAN is indicated to reduce blood phenylalanine (Phe) levels in adult and pediatric patients one month of age and older with hyperphenylalaninemia (HPA) due to tetrahydrobiopterin- (BH4-) responsive Phenylketonuria (PKU). KUVAN is to be used in conjunction with a Phe-restricted diet.

PCXX VANILLA ORANGE ONE MINUTE FM

Sodium Fluoride· Pascal

Detailed copay and financial assistance information is not publicly available for this medication at this time. Please consult your pharmacist or the manufacturer's official patient support program fo

Nulibry

fosdenopterin hydrobromide· Sentynl

* Patient Copay Amount: Not Publicly Available * Maximum Annual Benefit Limit: Not Publicly Available * Core Eligibility Restrictions: Must be a United States resident and have a prescription for a th

Clinical Trials

20 recruitingView all trials with filters →
Phase 34 trials
A Study of Sepiapterin in Participants With Phenylketonuria (PKU)
Phase 3
Actively Recruiting
PI: Patient Advocacy · Sites: Indianapolis, Indiana · Age: 010 yrs
Study to Evaluate the Safety and Efficacy of Pegvaliase in Adolescents (Ages 12-17) With Phenylketonuria
Phase 3
Active
PI: Study Director (BioMarin Pharmaceutical) · Sites: Phoenix, Arizona; Little Rock, Arkansas +14 more · Age: 1217 yrs
A Long-Term Safety Study of PTC923 in Participants With Phenylketonuria
Phase 3
Active
· Sites: Aurora, Colorado; Gainesville, Florida +18 more
A Study to Evaluate the Safety and Efficacy of JNT-517 in Participants With Phenylketonuria (PKU)
Phase 3
Actively Recruiting
PI: Otsuka Call Center · Sites: Los Angeles, California; Gainesville, Florida +18 more · Age: 1899 yrs
Phase 41 trial
Effect of L-carnitine Supplementation on Phenylalanine and Brain-derived Neurotrophic Factor Levels in Infants and Children With Phenylketonuria
Phase 4
Active
· Sites: Al Mansurah · Age: 118 yrs
Phase 15 trials
Evaluation of BH4 Responsiveness in Our PKU Patients
Phase 1
Active
· Sites: Sohag
Safety and Efficacy Study of NGGT002 in cPKU Adult Subjects
Phase 1
Actively Recruiting
PI: Jianping Weng, PhD (First Affiliated Hospital of Bengbu Medical Colleg) · Sites: Bengbu, Anhui; Shanghai · Age: 1855 yrs
AAV Gene Therapy Clinical Study in Adult Classic PKU (PHEdom)
Phase 1
Actively Recruiting Prior treatment eligible
PI: Study Contact · Sites: Orange, California; Minneapolis, Minnesota +3 more · Age: 1855 yrs
A Phase 1/Phase 2 Open-label Study to Evaluate the Safety, Tolerability, and Efficacy of a Single Intravenous Administration of SAR444836 in Adult Participants With Phenylketonuria
Phase 1
Active
PI: Clinical Sciences & Operations (Sanofi) · Sites: San Francisco, California; Aurora, Colorado +9 more · Age: 1865 yrs
First-in-Human, Multiple Part Clinical Study of JNT-517 in Healthy Participants and in Participants With Phenylketonuria
Phase 1
Active
· Sites: Gainesville, Florida; Tampa, Florida +13 more · Age: 1865 yrs
N/A9 trials
GMP Powdered Substitutes in PKU and TYR
N/A
Actively Recruiting
PI: Gary Hubbard, Dr (Nutricia UK Ltd) · Sites: Birmingham · Age: 399 yrs
A Long-term, Post-marketing Safety Study of Palynziq in Patients With PKU (PALace)
N/A
Actively Recruiting
PI: Medical Director, MD (BioMarin Pharmaceutical) · Sites: Aurora, Colorado; Chicago, Illinois +14 more
A Self-test Home-use Blood Phenylalanine Monitoring System Under the Brand Name Egoo Phe System Has Been Developed for Measurement of Phenylalanine (Phe) in Individuals Diagnosed With Phenylketonuria (PKU): The Study Purpose is to Evaluate Accuracy and Usability
N/A
Enrolling by Invitation
PI: Anita MacDonald (Birmingham Women's and Children's NHS Foundation T) · Sites: Birmingham · Age: 03 yrs
Long-Term Follow Up Study of Subjects Previously Administered HMI 102
N/A
Enrolling by Invitation
· Sites: Boston, Massachusetts; New York, New York · Age: 1855 yrs
A Long Term, Post-marketing Study of Immune Response in Patients Receiving Palynziq Treatment for PKU (PALisade)
N/A
Actively Recruiting
PI: Medical Director, MD (BioMarin Pharmaceutical) · Sites: Aurora, Colorado; Chicago, Illinois +4 more
Walking Program in Fatty Liver Children With Phenylketonuria
N/A
Actively Recruiting
PI: Ali Ismail, lecturer (Cairo University) · Sites: Dokki, Giza Governorate · Age: 912 yrs
Metabolic Control and Patient Well-being in Phenylketonuria: do Guidelines Make a Difference?
N/A
Actively Recruiting
PI: Martina Huemer, Prof · Sites: Zurich · Age: 1099 yrs
Brain Aging in Phenylketonuria
N/A
Actively Recruiting
PI: Regula Everts, Prof. Dr. phil. (Department of Diabetes, Endocrinology, Nutritional) · Sites: Bern · Age: 1899 yrs
Development of Novel Psychological Assessment Tools and Anxiety Intervention for Phenylketonuria
N/A
Actively Recruiting
PI: Shawn Christ · Sites: Columbia, Missouri · Age: 1017 yrs

Specialists

Showing 25 of 32View all specialists →
MP
Martina Huemer, Prof
Zurich
Specialist

Rare Disease Specialist

DM
Dorothy K. Grange, M.D.
SAINT LOUIS, MO
Specialist
PI on 1 active trial
DP
Desiree White, Ph.D.
Specialist
PI on 2 active trials
HD
Huan Zhou, Doctor
Bengbu, Anhui
Specialist

Rare Disease Specialist

OC
Otsuka Call Center
Los Angeles, California
Specialist

Rare Disease Specialist

4 Phenylketonuria publications
DL
Debra Lounsbury
Specialist
PI on 5 active trials1 Phenylketonuria publication
GD
Gary Hubbard, Dr
Birmingham
Specialist

Rare Disease Specialist

PI on 1 active trial
JM
Joshua Lilienstein, M.D.
JAMAICA PLAIN, MA
Specialist
PI on 1 active trial
AM
Anita MacDonald
Birmingham
Specialist

Rare Disease Specialist

PI on 10 active trials
RP
Rajavel Elango, PhD
Vancouver, British Columbia
Specialist

Rare Disease Specialist

PI on 5 active trials
SM
Suyash Prasad, MD
Specialist
PI on 1 active trial
AM
Annette Feigenbaum, MD
SAN DIEGO, CA
Specialist
PI on 1 active trial1 Phenylketonuria publication
RP
Rebecca Stratton, PhD
GREENVILLE, MI
Specialist
PI on 3 active trials
HM
Heather W Saavedra, MS
CHARLESTON, SC
Specialist
PI on 2 active trials
JP
Júlio C Rocha, PhD
Specialist
PI on 1 active trial
RP
Regula Everts, Prof. Dr. phil.
Bern
Specialist

Rare Disease Specialist

PI on 2 active trials
KR
Krista Viau, PhD, RD
SALT LAKE CITY, UT
Specialist
PI on 1 active trial
LM
Lori-Anne P Schillaci, MD
CLEVELAND, OH
Specialist
PI on 1 active trial1 Phenylketonuria publication
SC
Study Contact
Orange, California
Specialist

Rare Disease Specialist

PA
Patient Advocacy
Indianapolis, Indiana
Specialist

Rare Disease Specialist

PI on 2 active trials1 Phenylketonuria publication

Treatment Centers

8 centers
⚗️ Trial Site

Children's Hospital Colorado

📍 Aurora, Colorado

👤 Zachary Grinspan, MD

⚗️ Trial Site

Arkansas Children's Hospital

📍 Little Rock, Arkansas

⚗️ Trial Site

Boston Children's Hospital

📍 Boston, Massachusetts

⚗️ Trial Site

Children's Hospital of Philadelphia

📍 Philadelphia, Pennsylvania

👤 Study Director

👤 Richard Neibeger, MD

⚗️ Trial Site

Ann & Robert H. Lurie Children's Hospital of Chicago

📍 Chicago, Illinois

⚗️ Trial Site

Northwestern University

📍 Chicago, Illinois

👤 Ann (Annie) W Silk

⚗️ Trial Site

Vanderbilt University Medical Center

📍 Nashville, Tennessee

⚗️ Trial Site

University of Minnesota

📍 Minneapolis, Minnesota

Financial Resources

10 resources
Sephience(SEPIAPTERIN)PTC Therapeutics Inc.
Palynziq(PEGVALIASE-PQPZ)BioMarin Pharmaceutical Inc.
Sapropterin Dihydrochloride(SAPROPTERIN DIHYDROCHLORIDE)Aurobindo Pharma Limited
Kuvan(sapropterin)BioMarin Pharmaceutical, Inc.

Good Days — Phenylketonuria

Good Days

Phenylketonuria

Verified 3d ago
Foundation Grant
foundation grant
Accepting applications

Patient Services Inc — Phenylketonuria

Patient Services Inc

Phenylketonuria

Verified 3d ago
Foundation Grant
foundation grant
Accepting applications

NORD Patient Assistance — Phenylketonuria

NORD Patient Assistance

Phenylketonuria

Verified 3d ago
Foundation Grant
foundation grant
Accepting applications

HealthWell Foundation — Phenylketonuria

HealthWell Foundation

Phenylketonuria

Verified 3d ago
Foundation Grant
foundation grant
Accepting applications

The Assistance Fund — Phenylketonuria

The Assistance Fund

Phenylketonuria

Verified 3d ago
Foundation Grant
foundation grant
Accepting applications

PAN Foundation — Phenylketonuria

PAN Foundation

Phenylketonuria

Verified 3d ago
Foundation Grant
foundation grant
Currently closed
Apply Get notified when open

Travel Grants

No travel grants are currently matched to Phenylketonuria.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Phenylketonuria

3 articles
NewsUNITERAREApr 20, 2026
Fund Update: PAN Foundation — Phenylketonuria Phenylketonuria fund is currently closed
The PAN Foundation's financial assistance program for phenylketonuria (PKU) — a rare genetic condition where the body can't break down a protein called phenylal
ResearchPUBMEDApr 9, 2026
The Role of Digital Tools and Their Implementation Within Patient Care Pathways for Rare Brain Disorders: The Case of Phenylketonuria.
Researchers studied how digital tools like telemedicine and online apps can help patients with phenylketonuria (PKU), a rare genetic disorder affecting how the
NewsUNITERAREApr 3, 2026
Fund Update: PAN Foundation — Phenylketonuria Fund Phenylketonuria fund is currently closed
The PAN Foundation's financial assistance program for phenylketonuria (PKU) — a rare genetic condition that affects how the body processes a protein called phen
See all news about Phenylketonuria

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Common questions about Phenylketonuria

What is Phenylketonuria?

Phenylketonuria is treated with 8 medications in our database, including Sapropterin Dihydrochloride, SAPROPTERIN DIHYDROCHLORIDE, Sapropterin dihydrochloride, PCXX VANILLA ORANGE ONE MINUTE FM, Palynziq, and 3 more. 7 of these have manufacturer assistance programs available to help reduce out-of-pocket costs. Medications are manufactured by BioMarin, Pascal, Sentynl. Patients and caregivers can find copay cards, patient assistance programs, and travel grants for Phenylketonuria treatment below.

How is Phenylketonuria inherited?

Phenylketonuria follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Phenylketonuria typically begin?

Typical onset of Phenylketonuria is neonatal. Age of onset can vary across affected individuals.

Are there clinical trials for Phenylketonuria?

Yes — 20 recruiting clinical trials are currently listed for Phenylketonuria on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Phenylketonuria?

25 specialists and care centers treating Phenylketonuria are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.

What treatment and support options exist for Phenylketonuria?

5 FDA-approved treatments and 9 patient support programs are currently tracked on UniteRare for Phenylketonuria. See the treatments and support programs sections for copay assistance, eligibility, and contact details.