Sodium Phenylbutyrate · Buphenyl, Pheburane

What is Sodium Phenylbutyrate?

Sodium phenylbutyrate is a pro-drug metabolized to phenylacetate, which conjugates with glutamine to form phenylacetylglutamine for renal excretion. It provides an alternate pathway for waste nitrogen excretion in urea cycle disorders and is studied in medium-chain acyl-CoA dehydrogenase deficiency.

Summary written with AI from the sources listed at the end of this page.

At a glance

Also known as

Buphenyl, Pheburane, ACER-001, NSC-657802, Olpruva

Drug type

Small molecule

Pathways

nitrogen excretion, urea cycle

Therapy area

Metabolic

Developer

Horizon Therap Us

In trials of

ZVRA

FDA status

Approved as Buphenyl, Pheburane (April 30, 1996), NDA020573 · FDA label

UNII

NT6K61736T

✅ FDA-approved uses

• Urea cycle disorders (CPS, OTC, or AS deficiency) (Pheburane)
• Urea cycle disorders (CPS, OTC, or AS deficiency) (Buphenyl)

From the indications section of the FDA label (version of Jul 17, 2026). Read the full label for the exact wording, limitations and safety information.

🧪 Clinical trials

Trials of the companies BioRadar tracks, from ClinicalTrials.gov, most advanced phase first.

Conditions studied

Medium-chain Acyl-CoA Dehydrogenase Deficiency

Sources

• FDA label (DailyMed)
• FDA label Pheburane (2026-07-17), indications
• FDA label Buphenyl (2024-10-30), indications
• ClinicalTrials.gov NCT06773026

Updated Oct 3, 2026.