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Berotralstat

Phase 3

Hereditary Angioedema | Small molecule | Immunology |BioCryst Pharmaceuticals, Inc.|Last Updated: Feb 25, 2026

Target and mechanism

Molecular targetKLKB1
Target classInhibitor
ModalitySmall molecule

Success Probability

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Market & Valuation

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Trial Design

CONTROLLEDDMC
Total Trials2
Total Enrollment168

FDA Designations

No designations recorded

Clinical trial landscape

Berotralstat · 2 trials · 3 indications

Phase 3 2
NCT05453968Berotralstat Treatment in Children With Hereditary AngioedemaHereditary Angioedema
ACTIVE NOT_RECRUITING29 Analytics
NCT04933721Open-label Berotralstat Access to HAE Patients Previously Enrolled in Berotralstat StudiesHereditary Angioedema
ENROLLING BY_INVITATION139 Analytics
PHASE3ACTIVE NOT_RECRUITING
Berotralstat Treatment in Children With Hereditary Angioedema
Hereditary AngioedemaUnlock trial analytics
PHASE3ENROLLING BY_INVITATION
Open-label Berotralstat Access to HAE Patients Previously Enrolled in Berotralstat Studies
Hereditary AngioedemaUnlock trial analytics

Study Endpoints

Primary Endpoints

Area Under the Plasma Concentration-Time Curve From Time 0 to the Last Measurable Concentration (AUC0-last) of Berotralstat
Week 2

AUC0-last is the area under the plasma concentration-time curve from time 0 to the time of the last measurable concentration.

Area Under the Plasma Concentration-Time Curve From Time 0 to 6 Hours Post-dose (AUC0-6) of Berotralstat
Predose and up to 6 hours post dose at Week 2

AUC0-6 is the area under the plasma concentration-time curve from time 0 to 6 hours.

Concentration at the End of the Dosing Interval (Ctrough) of Berotralstat
Predose at Week 2

Ctrough is the concentration at the end of a dosing interval of berotralstat.

Maximum Observed Plasma Concentration (Cmax) of Berotralstat
Predose and up to 6 hours post dose at Week 2

Cmax is the maximum observed plasma concentration of berotralstat.

Time of Last Measurable Plasma Concentration (Tlast) of Berotralstat
Predose and up to 6 hours post dose at Week 2

Tlast is the time of the last measurable concentration (Clast) of berotralstat collected over the sampling interval.

Time to Maximum Plasma Concentration (Tmax) of Berotralstat
Predose and up to 6 hours post dose at Week 2

Tmax is the time taken to reach the maximum observed plasma concentration of berotralstat.

Number and proportion of subjects with a treatment-related TEAE
240 weeks
Number and proportion of subjects who experience a serious adverse event (SAE)
240 weeks
Number and proportion of subjects who experience a treatment-related Grade 3 or 4 TEAE
240 weeks
Number and proportion of subjects who experience a treatment-related Grade 3 or 4 treatment-emergent chemistry abnormality
240 weeks
Number and proportion of subjects who discontinue due to a TEAE
240 weeks

Secondary Endpoints

Number of Participants With Treatment Emergent Adverse Events (TEAEs)
From first dose of study treatment up to approximately 73 weeks
Number of Adjusted Hereditary Angioedema (HAE) Attacks
Week 1 through Week 12 and Week 1 through Week 48
Rate of Adjusted HAE Attacks
Week 1 through Week 12 and Week 1 through Week 48
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Study Design & Arms

AllocationNON_RANDOMIZED
MaskingNONE
ModelSINGLE_GROUP
PurposePREVENTION

Treatment Arms

ArmTypeDescription
Cohort 1: ≥ 40 kg body weight (Berotralstat 150 mg)EXPERIMENTALParticipants received 150 milligram (mg) berotralstat capsule orally once daily for up to 144 weeks. Dose modifications were permitted due to weight changes, PK results, or safety.
Cohort 2: 32 to < 40 kg body weight (Berotralstat 108 mg)EXPERIMENTALParticipants received 108 mg berotralstat granules orally once daily for up to 144 weeks. Dose modifications were permitted due to weight changes, PK results, or safety.
Cohort 3: 24 to < 32 kg body weight (Berotralstat 96 mg)EXPERIMENTALParticipants received 96 mg berotralstat granules orally once daily for up to 144 weeks. Dose modifications were permitted due to weight changes, PK results, or safety.
Cohort 4: 12 to <24 kg body weight (Berotralstat 78 mg)EXPERIMENTALParticipants received 78 mg berotralstat granules orally once daily for up to 144 weeks. Dose modifications were permitted due to weight changes, PK results, or safety.
BCX7353 capsules or granules once dailyEXPERIMENTALBerotralstat (BCX7353) capsules or granules orally administered once daily.

Interventions

NameTypeDescription
BerotralstatDRUGAdministered orally once daily
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Eligibility Criteria

Age Range2 Years to 11 Years
SexALL
Healthy VolunteersNo
Study Sites15

Inclusion Criteria: * Male and non-pregnant, non-lactating females 2 to \< 12 years of age * Body weight ≥ 12 kg * Clinical diagnosis of HAE * In the opinion of the investigator, the participant would benefit from long term oral HAE prophylaxis * For subjects who are not currently receiving prophyl...

Countries:AustriaCanadaFranceGermanyIsraelItalyPolandRomaniaSpainUnited KingdomCzechiaNorth MacedoniaSlovakiaSouth AfricaSouth Korea
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Frequently asked questions about Berotralstat

What is Berotralstat used for?

Berotralstat is an investigational small molecule being developed for the treatment of Hereditary Angioedema (HAE), a rare genetic condition characterized by recurrent episodes of severe swelling. It is currently in Phase 3 clinical development and has not yet been approved by regulatory authorities.

What does Berotralstat target?

Berotralstat targets and inhibits the KLKB1 protein, which plays a role in the kallikrein-kinin system. By inhibiting KLKB1, Berotralstat aims to reduce the production of bradykinin, a key mediator of swelling attacks in Hereditary Angioedema. This mechanism is being evaluated in clinical trials.

Who makes Berotralstat?

Berotralstat is being developed by BioCryst Pharmaceuticals, Inc., a biopharmaceutical company traded on the NASDAQ under the ticker symbol BCRX. The company is conducting Phase 3 clinical trials to evaluate the drug's safety and efficacy in patients with Hereditary Angioedema.

What phase is Berotralstat in?

Berotralstat is currently in Phase 3 clinical development for Hereditary Angioedema. It is an investigational drug, meaning it has not yet received regulatory approval and is still being studied in clinical trials to assess its safety and effectiveness in patients.

What clinical trials is Berotralstat in?

Berotralstat is being studied in two Phase 3 clinical trials. NCT04933721 is an open-label access study for HAE patients previously enrolled in Berotralstat studies, enrolling 139 participants. NCT05453968 is a study of Berotralstat treatment in children with Hereditary Angioedema, enrolling 29 pediatric participants.

Is Berotralstat being studied in pediatric patients?

Yes, Berotralstat is being studied in pediatric patients with Hereditary Angioedema. The clinical trial NCT05453968, titled 'Berotralstat Treatment in Children With Hereditary Angioedema,' is actively enrolling children as young as 2 years old across multiple countries, including Austria, Canada, France, Germany, and the United Kingdom.