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phenylbutyrate

Phase 2

Medium-chain Acyl-CoA Dehydrogenase Deficiency | Small molecule | Rare Disease |Zevra Therapeutics, Inc.|Last Updated: Aug 15, 2025

Target and mechanism

ModalitySmall molecule

Also known as Sodium phenylbutyrate

Success Probability

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Market & Valuation

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Trial Design

UNCONTROLLEDBiomarker
Total Trials1
Total Enrollment24

FDA Designations

No designations recorded

Clinical trial landscape

phenylbutyrate · 2 trials · 2 indications

Phase 2 1Phase 1 1
NCT06773026Study of Sodium Phenylbutyrate (ACER-001) for the Treatment of Pediatric and Adults Patients With Medium Chain Acyl-CoA Dehydrogenase Deficiency (MCADD)Medium-chain Acyl-CoA Dehydrogenase Deficiency
RECRUITING24 Analytics
PHASE2RECRUITING
Study of Sodium Phenylbutyrate (ACER-001) for the Treatment of Pediatric and Adults Patients With Medium Chain Acyl-CoA Dehydrogenase Deficiency (MCADD)
Medium-chain Acyl-CoA Dehydrogenase DeficiencyUnlock trial analytics

Study Endpoints

Primary Endpoints

Number of participants with treatment related adverse events as assessed by CTCAE v5.0
5 weeks
Number of treatment related adverse events
2 years

Secondary Endpoints

Length of time before glucose falls below 60 mg/dL
5 weeks
Length of time before glucose falls below 70 mg/dL
5 weeks
Length of time before glucose falls below 80 mg/dL
5 weeks
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Study Design & Arms

AllocationNA
MaskingNONE
ModelSINGLE_GROUP
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
4.0 g/m2/day BID sodium phenylbutyrateEXPERIMENTALUp to 24 subjects (12: ages 4-9 years old; 12:10 years of age and older) will be randomized to take 4.0 g/m2/day divided into two daily doses
sodium phenylbutyrateEXPERIMENTALa single patient will take sodium phenylbutyrate (ACER-001) current at a dose of 12 g/day; dose will be modified if the patient gains more than 5 kg of weight

Interventions

NameTypeDescription
Sodium phenylbutyrateDRUGOpen-label design with doses of sodium phenylbutyrate at 4.0 g/m2/day
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Eligibility Criteria

Age Range4 Years to N/A
SexALL
Healthy VolunteersNo
Study Sites1

Inclusion Criteria: 1. A diagnosis of MCADD and molecular confirmation of at least one copy of the common c.985A\>G mutation. 2. ≥4 years of age 3. Able to perform and comply with study activities placement of a continuous glucose monitor, IV catheter, and all blood draws. 4. Negative pregnancy tes...

Countries:United States
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Frequently asked questions about phenylbutyrate

What is phenylbutyrate used for?

Phenylbutyrate, also known as sodium phenylbutyrate, is an investigational small molecule being studied for the treatment of Medium-chain Acyl-CoA Dehydrogenase Deficiency (MCADD) and Combined D,L-2-hydroxyglutaric Aciduria (C-2HGA). It is currently in clinical development and has not been approved for these indications.

Who makes phenylbutyrate?

Phenylbutyrate is being developed by Zevra Therapeutics, Inc. (NASDAQ: ZVRA). The company is conducting clinical trials to evaluate the drug for the treatment of Medium-chain Acyl-CoA Dehydrogenase Deficiency and Combined D,L-2-hydroxyglutaric Aciduria.

What phase is phenylbutyrate in?

Phenylbutyrate is in Phase 1 and Phase 2 clinical trials. A Phase 2 study is recruiting patients with Medium-chain Acyl-CoA Dehydrogenase Deficiency, and a Phase 1 study is enrolling by invitation for Combined D,L-2-hydroxyglutaric Aciduria. The drug is investigational and not yet approved.

What clinical trials is phenylbutyrate in?

Phenylbutyrate is being studied in two clinical trials. NCT06773026 is a Phase 2 study of sodium phenylbutyrate (ACER-001) for pediatric and adult patients with Medium-chain Acyl-CoA Dehydrogenase Deficiency, with an enrollment of 24 participants. NCT07125066 is a Phase 1 open-label study for Combined D,L-2-hydroxyglutaric Aciduria with one participant.

Is phenylbutyrate the same as sodium phenylbutyrate?

Yes, phenylbutyrate is also known as sodium phenylbutyrate. In clinical trials, it is referred to as ACER-001. The drug is being investigated for the treatment of Medium-chain Acyl-CoA Dehydrogenase Deficiency and Combined D,L-2-hydroxyglutaric Aciduria.