Drugs / Betaine
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Betaine

Small molecule targets PEX1 via restoration

Developed for
Angelman syndrome · chronic hepatitis C virus infection · female reproductive system disorder · hepatitis C virus infection · homocystinuria · hyperhomocysteinemia · major depressive disorder · male infertility
+6 more · metabolic dysfunction-associated steatohepatitis · peroxisome biogenesis disorder · primary hyperoxaluria type 1 · syringomyelia · glucose metabolism disease · obesity disorder
Investigated by
University of California, San Francisco · Assistance Publique - Hôpitaux de Paris · Baylor College of Medicine · Boston Children's Hospital · Cambridge Health Alliance · Center for Health Sciences, Serbia
+20 more · Children's Hospital and Medical Center, Omaha, Nebraska · Columbia University · Genentech, Inc. · Greenwood Genetic Center · Instituto de Investigación Sanitaria de la Fundación Jiménez Díaz · Lumenis Be Ltd. · Mayo Clinic · McGill University Health Centre/Research Institute of the McGill University Health Centre · Mucosa Innovations, S.L. · National Alliance for Research on Schizophrenia and Depression · National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) · Orphan Europe · Rady Children's Hospital, San Diego · Rare Diseases Clinical Research Network · Southern California Institute for Research and Education · University Hospital, Basel, Switzerland · University of California, San Diego · University of Nebraska · University of North Carolina, Chapel Hill · Xuanwu Hospital, Beijing

Regulatory milestones approvals, filings & regulatory actions · 2 recorded

MilestoneJurisdictionBrandIndicationDateSentence it was read from
Approved EU (EMA) Cystadane 2007-02-14 europa.eu
Approved US (FDA) CYSTADANE 1996-10-25 fda.gov

Trials 15 · a red edge is where a trial was stopped

PhaseRegistry idDatesIndicationSponsorStatusOutcome
Phase 01 trial
Phase 0 NCT00882193 May 2009 → Jan 2011 chronic hepatitis C virus infection University of Nebraska Terminated No outcome recorded
Phase 13 trials
Phase 1 NCT02122211 Apr 2014 → Jul 2016 male infertility University of North Carolina, Chapel Hill Completed No outcome recorded
Phase 1 NCT01759875 Jan 2013 → Jan 2014 University of California, San Francisco Completed No outcome recorded
Phase 1 NCT01398046 Aug 2011 → Feb 2014 University of California, San Francisco Completed No outcome recorded
Phase 24 trials
Phase 2 NCT07276204 Mar 2026 → Dec 2029 expected metabolic dysfunction-associated steatohepatitis Southern California Institute for Research and Education Not yet recruiting No outcome recorded
Phase 2 NCT06308367 Mar 2024 → Mar 2027 expected syringomyelia Xuanwu Hospital, Beijing Recruiting No outcome recorded
Phase 2 NCT02404337 Jul 2015 → Dec 2017 homocystinuria Assistance Publique - Hôpitaux de Paris Completed No outcome recorded
Phase 2 NCT00283387 Feb 2007 → Sep 2011 primary hyperoxaluria type 1 Mayo Clinic Completed No outcome recorded
Phase 2/31 trial
Phase 2/3 NCT00310336 Aug 2006 → Aug 2009 hepatitis C virus infection University Hospital, Basel, Switzerland Completed No outcome recorded
Phase 32 trials
Phase 3 NCT01838941 Mar 2013 → Jan 2015 peroxisome biogenesis disorder McGill University Health Centre/Research Institute of the McGill University Health Centre Completed No outcome recorded
Phase 3 NCT01371357 May → Dec 2011 hyperhomocysteinemia Center for Health Sciences, Serbia Completed No outcome recorded
Phase 42 trials
Phase 4 NCT01749982 Dec 2012 → Mar 2013 Columbia University Completed No outcome recorded
Phase 4 NCT00226356 Dec 2004 → Jun 2007 major depressive disorder Cambridge Health Alliance Completed No outcome recorded
Phase not applicable2 trials
NCT05585476 Oct 2025 → Jun 2026 overdue female breast carcinoma, female reproductive system disorder Instituto de Investigación Sanitaria de la Fundación Jiménez Díaz Not yet recruiting No outcome recorded
NCT00348933 Jul 2006 → Feb 2010 Angelman syndrome University of California, San Diego Completed No outcome recorded
Also used as a comparator or background therapy in 3 trials
PhaseRegistry idDatesIndicationSponsorStatusOutcome
Phase 22 trials
Phase 2 NCT01950039 Comparator Jan 2014 → Dec 2017 glucose metabolism disease, obesity disorder Joslin Diabetes Center Completed No outcome recorded
Phase 2 NCT00586911 Comparator Jan 2003 → Jun 2006 metabolic dysfunction-associated steatohepatitis Mayo Clinic Completed No outcome recorded
Phase not applicable1 trial
NCT00571714 Comparator Apr 2008 → Mar 2010 chronic hepatitis C virus infection University of Nebraska Withdrawn No outcome recorded

Evidence & citations 19 cited values

Every value below carries the sentence it was read from. 20 sources stand behind the page.

FieldValueCited text
Known as Betaine “different dietary agents were used in the past for the treatment of elevated total plasma homocysteine (T-HCy), e. g. betaine, choline (betaine precursor) or folic acid.” NCT01371357
13

NCT00882193

NCT00283387

NCT01838941

NCT01749982

NCT06308367

NCT00226356

NCT02122211

NCT00571714

NCT00310336

NCT00348933

NCT07276204

NCT01950039

NCT02404337

Known as Abromine ChEMBL registry synonym — accepted as the source's own label CHEMBL95889
Known as Acidol pepsin ChEMBL registry synonym — accepted as the source's own label CHEMBL95889
Known as Amversio ChEMBL registry synonym — accepted as the source's own label CHEMBL95889
Known as anhydrous betaine HCl “This pilot study evaluated the extent and time course of gastric reacidification after a solid oral dosage form of anhydrous betaine HCl in healthy volunteers with...” PMID 23980906 Sep 2013
Known as Betaine anhydrous ChEMBL registry synonym — accepted as the source's own label CHEMBL95889
Known as betaine HCl “After gastric pH > 4 was confirmed for 15 min, 1500 mg of betaine HCl was given orally with 90 mL of water and gastric pH was continuously monitored for 2 h.” PMID 23980906 Sep 2013
Known as Betaine Hydrochloride ClinicalTrials.gov intervention name — accepted as the source's own label NCT01398046
1

NCT01759875

Known as Betainum muriaticum ChEMBL registry synonym — accepted as the source's own label CHEMBL95889
Known as Cystadane ClinicalTrials.gov intervention name — accepted as the source's own label NCT00586911
Known as FEMA NO. 4223 ChEMBL registry synonym — accepted as the source's own label CHEMBL95889
Known as NSC-166511 ChEMBL registry synonym — accepted as the source's own label CHEMBL95889
Known as NSC-32191 ChEMBL registry synonym — accepted as the source's own label CHEMBL95889
Known as NSC-3976 ChEMBL registry synonym — accepted as the source's own label CHEMBL95889
Known as trimethylglycine “Betaine, or trimethylglycine, is a Health Canada and FDA approved orphan drug for the treatment of homocystinuria” NCT01838941
1

“It is a moisturizing and regenerating gel whose active ingredients are olive oil, trimethylglycine and xylitol.” NCT05585476

Action Restore “we reported the recovery of peroxisome functions by treatment with Betaine” NCT01838941
Modality Small molecule CHEMBL95889
Route Oral “Subjects were randomly assigned oral betaine 12 grams/day in subjects younger than 10 years of age, and 20 grams/day in subjects 10 years of age and older, in two divided doses.” NCT00283387
Target PEX1 “acting as a nonspecific chemical chaperone for the misfolded PEX1 protein” NCT01838941