Drugs / Sodium Phenylbutyrate
last change Jun 2026 re-read 2 minutes ago

Sodium Phenylbutyrate

Small molecule

Developed for
amyotrophic lateral sclerosis · leukemia · lymphoma · myelodysplastic syndrome · type 2 diabetes mellitus · brain neoplasm · medium chain acyl-CoA dehydrogenase deficiency · myelodysplastic/myeloproliferative disease
+31 more · urea cycle disorder · alpha 1-antitrypsin deficiency · argininosuccinic aciduria · D,L-2-hydroxyglutaric aciduria · diabetes mellitus · gastric cancer · head and neck cancer · Huntington disease · inborn disorder of amino acid metabolism · inclusion body myositis · lung cancer · lymphoproliferative syndrome · Machado-Joseph disease · MYO5B-related progressive familial intrahepatic cholestasis · neuroblastoma · obesity disorder · plasma cell myeloma · prostate cancer · proteinuria · pyruvate dehydrogenase deficiency · small intestine cancer · spinal muscular atrophy, type 1 · spinal muscular atrophy, type II · spinal muscular atrophy, type III · type 1 diabetes mellitus · Alzheimer disease · cystic fibrosis · HIV infectious disease · maple syrup urine disease · pulmonary tuberculosis · spinal muscular atrophy
Investigated by
National Cancer Institute (NCI) · Amylyx Pharmaceuticals Inc. · Jerry Vockley, MD, PhD · Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins · Amgen · Johns Hopkins University
+39 more · Massachusetts General Hospital · National Heart, Lung, and Blood Institute (NHLBI) · National Institute of Neurological Disorders and Stroke (NINDS) · University of California, San Diego · Westat · Zevra Therapeutics · Acer Therapeutics Inc. · Alpha-1 Foundation · Assistance Publique - Hôpitaux de Paris · Brantly, Mark L., M.D. · Breakthrough T1D · Brendan Lee · Columbia University · Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) · Fondazione Telethon · Fundação de Amparo à Pesquisa do Estado do Rio Grande do Sul, Brazil · HP Therapeutics Foundation · Hospital de Clinicas de Porto Alegre · Liminal BioSciences Ltd. · Maastricht University · Memorial Sloan Kettering Cancer Center · Muscular Dystrophy Association · National Center for Research Resources (NCRR) · Office of Rare Diseases (ORD) · Rare Diseases Clinical Research Network · URC-CIC Paris Descartes Necker Cochin · US Department of Veterans Affairs · University Health Network, Toronto · University of Alabama at Birmingham · University of Florida · University of Iowa · University of Kansas · University of Kansas Medical Center · University of Nottingham · University of Pennsylvania · University of Pittsburgh · University of Rochester · University of Washington · Washington University School of Medicine

Regulatory milestones approvals, filings & regulatory actions · 4 recorded

MilestoneJurisdictionBrandIndicationDateSentence it was read from
Label expansion US (FDA) OLPRUVA 2025-10-10 fda.gov
Refused (EMA) EU (EMA) Albrioza 2024-01-05 europa.eu
Approved EU (EMA) Ammonaps 1999-12-07 europa.eu
Approved US (FDA) BUPHENYL 1996-04-30 fda.gov

Trials 37 · a red edge is where a trial was stopped

PhaseRegistry idDatesIndicationSponsorStatusOutcome
Phase 01 trial
Phase 0 NCT06645769 Jun 2026 → Jan 2028 expected diabetes mellitus University of Pennsylvania Not yet recruiting No outcome recorded
Phase 18 trials
Phase 1 NCT07125066 Jul 2025 → Jul 2027 expected D,L-2-hydroxyglutaric aciduria Jerry Vockley, MD, PhD Enrolling by invitation No outcome recorded
Phase 1 NCT05349435 May → Jun 2022 urea cycle disorder or inherited hyperammonemia Liminal BioSciences Ltd. Terminated No outcome recorded
Phase 1 NCT04421677 Aug 2020 → Jan 2022 inclusion body myositis University of Kansas Medical Center Completed No outcome recorded
Phase 1 NCT00006239 Dec 2000 → Feb 2005 leukemia, myelodysplastic syndrome, myelodysplastic/myeloproliferative disease Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins Completed No outcome recorded
Phase 1 NCT00004871 May 2000 → ? leukemia, myelodysplastic syndrome, myelodysplastic/myeloproliferative disease Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins Completed No outcome recorded
Phase 1 NCT00005639 Mar 2000 → Jul 2005 lymphoma, small intestine cancer Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins Completed No outcome recorded
Phase 1 NCT00002909 Feb 1997 → Oct 2000 lymphoma National Cancer Institute (NCI) Completed No outcome recorded
Phase 1 NCT00001565 Dec 1996 → Oct 2000 brain neoplasm, neuroblastoma National Cancer Institute (NCI) Completed No outcome recorded
Phase 1/24 trials
Phase 1/2 NCT04987671 Aug 2021 → May 2022 amyotrophic lateral sclerosis Amylyx Pharmaceuticals Inc. Unknown No outcome recorded
Phase 1/2 NCT00439569 Jan → Aug 2008 spinal muscular atrophy, type II, spinal muscular atrophy, type III Westat Terminated No outcome recorded
Phase 1/2 NCT00439218 Jan 2008 → May 2009 spinal muscular atrophy, type 1 Westat Terminated No outcome recorded
Phase 1/2 NCT00107770 Apr 2005 → Nov 2006 amyotrophic lateral sclerosis US Department of Veterans Affairs Completed No outcome recorded
Phase 215 trials
Phase 2 NCT07699380 Jun 2026 → Dec 2029 expected type 1 diabetes mellitus University of Washington Recruiting No outcome recorded
Phase 2 NCT06773026 Jun 2025 → Jan 2027 expected medium chain acyl-CoA dehydrogenase deficiency Jerry Vockley, MD, PhD Recruiting No outcome recorded
Phase 2 NCT06069375 Apr 2024 → Jun 2028 medium chain acyl-CoA dehydrogenase deficiency Jerry Vockley, MD, PhD Suspended No outcome recorded
Phase 2 NCT03734263 Oct 2018 → Jul 2019 pyruvate dehydrogenase deficiency Fondazione Telethon Completed No outcome recorded
Phase 2 NCT03488524 Mar 2018 → Mar 2021 amyotrophic lateral sclerosis Amylyx Pharmaceuticals Inc. Completed No outcome recorded
Phase 2 NCT01096095 Jun → Dec 2010 Machado-Joseph disease Hospital de Clinicas de Porto Alegre Withdrawn No outcome recorded
Phase 2 NCT00947544 Mar 2010 → Aug 2011 urea cycle disorder Amgen Completed No outcome recorded
Phase 2 NCT00345605 Feb 2008 → May 2011 argininosuccinic aciduria Brendan Lee Completed No outcome recorded
Phase 2 NCT00387530 May 2006 → Jan 2008 gastric cancer, head and neck cancer, lymphoma, lymphoproliferative syndrome University of California, San Diego Withdrawn No outcome recorded
Phase 2 NCT00212316 Aug 2005 → Jun 2006 Huntington disease University of Rochester Completed No outcome recorded
Phase 2/32 trials
Phase 2/3 NCT04516096 Nov 2020 → Dec 2022 amyotrophic lateral sclerosis Amylyx Pharmaceuticals Inc. Completed No outcome recorded
Phase 2/3 NCT02343094 Feb → Dec 2015 proteinuria Assistance Publique - Hôpitaux de Paris Completed No outcome recorded
Phase 32 trials
Phase 3 NCT05619783 Dec 2022 → Oct 2024 amyotrophic lateral sclerosis Amylyx Pharmaceuticals Inc. Completed No outcome recorded
Phase 3 NCT00992459 Oct 2009 → Sep 2010 urea cycle disorder Amgen Completed No outcome recorded
Phase 42 trials
Phase 4 NCT05836350 Jun 2023 → Oct 2025 type 2 diabetes mellitus Maastricht University Unknown No outcome recorded
Phase 4 NCT00533559 Sep 2007 → Dec 2009 type 2 diabetes mellitus University Health Network, Toronto Completed No outcome recorded
Phase not applicable2 trials
NCT05028803 Sep 2021 → Sep 2023 University of Nottingham Completed No outcome recorded
NCT00771901 Feb 2008 → Dec 2014 obesity disorder, type 2 diabetes mellitus Washington University School of Medicine Completed No outcome recorded
Phase not stated1 trial
NCT01784718 MYO5B-related progressive familial intrahepatic cholestasis University of Pittsburgh No longer available No outcome recorded
Also used as a comparator or background therapy in 13 trials
PhaseRegistry idDatesIndicationSponsorStatusOutcome
Phase 11 trial
Phase 1 NCT00977600 Comparator Mar → Jul 2005 Amgen Completed No outcome recorded
Phase 1/24 trials
Phase 1/2 NCT00528268 Comparator Jul 2007 → Dec 2013 spinal muscular atrophy University of Utah Completed No outcome recorded
Phase 1/2 NCT00590538 Comparator Feb 2003 → Dec 2008 cystic fibrosis Children's Hospital of Philadelphia Terminated No outcome recorded
Phase 1/2 NCT00016744 Comparator Sep 2001 → Oct 2005 cystic fibrosis Children's Hospital of Philadelphia Completed No outcome recorded
Phase 1/2 NCT00002796 Background May 1997 → Dec 2004 colon mucinous adenocarcinoma, colon signet ring cell adenocarcinoma, rectal signet ring cell adenocarcinoma, rectum mucinous adenocarcinoma National Cancer Institute (NCI) Terminated No outcome recorded
Phase 25 trials
Phase 2 NCT03533257 Comparator Sep 2018 → Oct 2020 Alzheimer disease Amylyx Pharmaceuticals Inc. Completed No outcome recorded
Phase 2 NCT01702974 Comparator Sep 2012 → Aug 2015 HIV infectious disease Karolinska Institutet Completed No outcome recorded
Phase 2 NCT01698476 Comparator Sep 2012 → Aug 2015 pulmonary tuberculosis Karolinska Institutet Completed No outcome recorded
Phase 2 NCT01580007 Comparator Dec 2010 → Dec 2014 pulmonary tuberculosis International Centre for Diarrhoeal Disease Research, Bangladesh Completed No outcome recorded
Phase 2 NCT00551200 Comparator Oct 2007 → Jul 2008 urea cycle disorder Amgen Completed No outcome recorded
Phase 2/31 trial
Phase 2/3 NCT01529060 Comparator Feb 2013 → Sep 2017 maple syrup urine disease Brendan Lee Completed No outcome recorded
Phase 41 trial
Phase 4 NCT03335488 Comparator Feb 2018 → Jul 2022 urea cycle disorder Amgen Completed No outcome recorded
Phase not applicable1 trial
NCT02111200 Comparator Sep 2014 → Oct 2015 urea cycle disorder Baylor College of Medicine Completed No outcome recorded

Evidence & citations 15 cited values

Every value below carries the sentence it was read from. 51 sources stand behind the page.

FieldValueCited text
Known as sodium phenylbutyrate “The purpose of this study is to evaluate the safety, tolerability and clinical impact of 15-grams daily of sodium phenylbutyrate (phenylbutyrate) in Huntington's disease and to...” NCT00212316
30

“We, recently, showed that stimulating BCAA oxidation for 2 weeks with sodium-phenylbutyrate (NaPB) treatment -a drug known to accelerate BCAA oxidation- decreased BCAA plasma...” NCT05836350

“Our study seeks to investigate the potential small molecule inhibition of the kinase that regulates BCKDC by applying a novel activity of sodium phenylbutyrate (NaPBA), in MSUD.” NCT01529060

“Dose of interventions: 5,000 IU of vitamin D (cholecalciferol tablets) once daily and 500 mg PBA (sodium phenylbutyrate tablets) twice daily for 16 weeks.” NCT01698476

“Dose of interventions: 5,000 IU of vitamin D (cholecalciferol tablets) once daily and 500 mg PBA (sodium phenylbutyrate tablets) twice daily for 16 weeks.” NCT01702974

“The purpose of this study is to determine whether sodium phenylbutyrate can reduce Lcn2 urinary expression in proteinuric patients.” NCT02343094

“Oral sodium phenylbutyrate (Buphenyl®) equivalent to 3 g/m2 of 4-phenylbutyric acid (PBA) per dose” NCT00977600

“AMX0035 twice daily--a combination of Sodium Phenylbutyrate (3g) and Taurursodiol (1g)” NCT03533257

“Tauroursodeoxycholic Acid and Sodium Phenylbutyrate orally given twice a day” NCT04516096

“oral AMX0035 (sodium phenylbutyrate + taurursodiol) versus placebo” NCT07699380

“Fixed dose combination of Sodium Phenylbutyrate and taurursodiol.” NCT04987671

Sodium Phenylbutyrate (3g) and Taurursodiol (1g)” NCT03488524

“oral sodium phenylbutyrateNCT00002909

NCT00528268

NCT00345605

NCT00107770

NCT00004767

NCT00006019

NCT00002796

NCT01096095

NCT06069375

NCT06773026

NCT02111200

NCT05028803

NCT01580007

NCT05349435

NCT07125066

NCT00771901

NCT06645769

NCT00533559

NCT00439569

Known as 4-phenylbutyrate “This is a single patient compassionate use protocol to determine whether Buphenyl (4-phenylbutyrate) will improve the poor bile flow associated with Byler Disease.” NCT01784718
Known as 4-phenylbutyric acid, sodium salt “500 mg sodium phenylbutyrate (4-phenylbutyric acid, sodium salt) in tablet form twice daily and 5000 IU of cholecalciferol once daily will be given orally for 2 months” NCT01580007
Known as 4PBA “Sodium 4-Phenylbutyrate (4PBA)” NCT00016744
1

NCT00067756

Known as ACER-001 “The medication is sodium phenylbutyrate (ACER-001), which is currently FDA approved for the treatment of Urea Cyle Disorders.” NCT06069375
Known as Buphenyl Buphenyl is currently approved for the treatment of rare inherited disorders of the urea cycle.” NCT00533559
4

NCT00977600

NCT01784718

NCT00992459

NCT00551200

Known as Buphenyl-TM “Sodium phenylbutyrate (Buphenyl-TM) is a drug that has been used to treat other types of urea cycle disorders.” NCT00345605
Known as NaPB “The purpose of the study is to evaluate the safety of sodium phenylbutyrate (NaPB) treatment in subjects with amyotrophic lateral sclerosis (ALS) and the ability to take this...” NCT00107770
2

“This study will investigate if the drug sodium phenylbutyrate (NaPB) impacts blood pressure and vascular function in healthy volunteers and in patients with diabetes.” NCT06645769

“This has led us to try an alternative method, which is to lower BCAA levels using Sodium Phenylbutyrate (NaPB).” NCT05028803

Known as NaPBA “Our study seeks to investigate the potential small molecule inhibition of the kinase that regulates BCKDC by applying a novel activity of sodium phenylbutyrate (NaPBA), in MSUD.” NCT01529060
3

“Buphenyl (NaPBA) will be the comparator drug to HPN-100 in this study.” NCT00992459

NCT00947544

NCT03335488

Known as PBA “Dose of interventions: 5,000 IU of vitamin D (cholecalciferol tablets) once daily and 500 mg PBA (sodium phenylbutyrate tablets) twice daily for 16 weeks.” NCT01702974
1

“Immunotherapy using vitamin D (vitD3 ) and phenylbutyrate (PBA) may support standard drug regimens used to treat infectious diseases.” PMID 29696707 May 2018

Known as phenylbutyrate “500 mg sodium phenylbutyrate (4-phenylbutyric acid, sodium salt) in tablet form twice daily and 5000 IU of cholecalciferol once daily will be given orally for 2 months” NCT01580007
16

“Combination of 3 g phenylbutyrate and 1 g taurursodiol” NCT05619783

NCT00002909

NCT01702974

NCT00005639

NCT00212316

NCT00006240

NCT00006239

NCT01698476

NCT04421677

NCT00001565

NCT00006450

NCT03734263

NCT00004871

NCT01529060

NCT00387530

NCT00439218

Known as Sodium 4-Phenylbutyrate ClinicalTrials.gov intervention name — accepted as the source's own label NCT00016744
1

NCT00590538

Action Restore “Both drugs may be able to restore normal chloride movements in body organs and glands.” NCT00016744
Modality Small molecule “Phenylbutyrate is an aromatic fatty acid” NCT00006450
Route Intravenous “phenylbutyrate administered as a continuous intravenous infusion for 28 days” NCT00001565