Clinical Trials

Numerous clinical trials spanning Phase I through Phase III evaluate nitisinone for metabolic and rare genetic conditions, primarily Hereditary Tyrosinemia Type I and Alkaptonuria, alongside albinism, lactic acidosis, and drug-drug interactions in healthy volunteers. Sponsored by commercial entities such as Swedish Orphan Biovitrum and Cycle Pharmaceuticals alongside academic institutions including the University of Liverpool and NHGRI, these studies investigate safety, efficacy, and dosing regimens. Overall recruitment statuses for these trials include completed, unknown, and withdrawn.

NCT Number Recruitment Conditions Sponsor/Collaborators Start Date Phases
NCT04113772 UNKNOWN
Hereditary Tyrosinemia, Type I
Sutphin Drugs
2019-11-01
NCT03103568 COMPLETED
Drug Drug Interaction
Swedish Orphan Biovitrum
2017-03-28 PHASE1
NCT01838655 COMPLETED
Albinism; Vision Loss
National Eye Institute (NEI)
2013-04-16 PHASE1; PHASE2
NCT01390077 COMPLETED
Alkaptonuria
University of California, San Diego
2011-01 PHASE2; PHASE3
NCT02750345 COMPLETED
Hereditary Tyrosinemia, Type I
Cycle Pharmaceuticals Ltd.
2016-03 PHASE1
NCT01916382 UNKNOWN
Alkaptonuria
University of Liverpool
2014-04 PHASE3
NCT02750709 COMPLETED
Hereditary Tyrosinemia, Type I
Cycle Pharmaceuticals Ltd.
2015-10 PHASE1
NCT02750332 COMPLETED
Hereditary Tyrosinemia, Type I
Cycle Pharmaceuticals Ltd.
2015-11 PHASE1
NCT02323529 COMPLETED
Hereditary Tyrosinemia, Type I
Swedish Orphan Biovitrum
2014-12 PHASE3
NCT02323529 Completed
Hereditary Tyrosinemia Type I
Swedish Orphan Biovitrum
2014-12 Phase 3
NCT01828463 COMPLETED
Alkaptonuria
University of Liverpool
2013-05 PHASE2
NCT01857362 COMPLETED
Healthy
Swedish Orphan Biovitrum
2013-05 PHASE1
NCT01828463 Completed
Alkaptonuria
University of Liverpool|Liverpool University Hospitals NHS Foundation Trust
2013-05 Phase 2
NCT01734889 COMPLETED
Hereditary Tyrosinemia, Type I
Swedish Orphan Biovitrum
2012-10 PHASE1
NCT01682538 COMPLETED
Healthy Volunteers
Swedish Orphan Biovitrum
2012-08 PHASE1
NCT00107783 COMPLETED
Alkaptonuria
National Human Genome Research Institute (NHGRI)
2005-01 PHASE2
NCT00031161 COMPLETED
Acidosis, Lactic; Chronic Disease
University of Florida
2001-09

(data from https://clinicaltrials.gov, updated on 2019-10-03)

Check the NTBC (Nitisinone) product page for in-depth specifications, including solubility, stock solutions, MOA, and working concentrations.

Compliance for Clinical Use

Mechanism and Biochemical Profile

Nitisinone functions as a synthetic reversible inhibitor of 4-hydroxyphenylpyruvate dioxygenase, preventing the enzymatic conversion of 4-hydroxyphenylpyruvate to homogentisic acid within the tyrosine catabolic pathway. By suppressing the downstream accumulation of toxic metabolic intermediates, this biochemical blockade mitigates cellular toxicity and organ damage in clinical conditions such as hereditary tyrosinemia type I and alkaptonuria.

Appendix RUO and cGMP Quality Standards

Quality Dimension RUO (Research Use Only) cGMP (Current Good Manufacturing Practice)
Clinical Applicability Prohibited in human clinical trials or medical diagnostics. Mandatory for human clinical trials (Phase I–III) and therapies.
Regulatory Status Non-regulated grade; exempt from drug manufacturing laws. Legally enforced by health authorities (e.g., FDA, EMA, NMPA).
Facility Environment Unclassified analytical or research laboratories. Validated Cleanrooms (ISO Class 5–8) with continuous monitoring.
Quality Control Basic purity and activity testing. Rigorous safety release testing (Sterility, Endotoxin, Mycoplasma).
Process Validation Basic equipment calibration; no process validation required. Full qualification (IQ/OQ/PQ) and complete batch records.
Quality Assurance Vendor self-declared without required formal QMS. Mandatory QA/QC unit, Change Control, CAPA, and vendor audits.
Regulatory Impact High risk of IND rejection if used as a critical raw material. Required for IND/NDA filings, supported by Drug Master Files (DMF).

Footnotes

Regulatory Note: Governed by FDA (21 CFR Parts 210/211/312), EMA (EudraLex Vol 4), ICH Guidelines (E6/Q7/Q9/Q10), and compendial standards (USP <71>/<85>/<1043>).

Note: Technical data last updated: Sep 1, 2026.