TheraRadar
Data updated: May 26, 2026

NITISINONE

Hydroxyphenylpyruvate Dioxygenase Inhibitors
Rare Disease Approved 2019-08-26

NITISINONE is indicated for the treatment of Hereditary Tyrosinemia Type 1.

Source: FDA Label • ETON • 4-Hydroxyphenyl-Pyruvate Dioxygenase Inhibitor

How NITISINONE Works

This drug works by blocking an enzyme called 4-hydroxyphenyl-pyruvate dioxygenase, which is part of the process the body uses to break down tyrosine. By stopping this process early, it prevents the formation of harmful metabolic byproducts that cause organ damage and neurological issues. This helps protect the liver and kidneys while also preventing the porphyric crises associated with the disease.

Source: FDA Label
4
Indications
--
Phase 3 Trials
6
Years on Market

Details

Status
Prescription
First Approved
2019-08-26
Routes
ORAL
Dosage Forms
CAPSULE

NITISINONE Approval History

2020
2021
2022
2023
2024
2025
2026
Original
New Indication
New Form
Label Update
18 FDA actions from 2019 to 2025
Jul 2025 SUPPL
Label · Labeling
May 2023 ORIGINAL
Update
Jan 2023 ORIGINAL
Update

What NITISINONE Treats

1 indications

NITISINONE is approved for 1 conditions since its original approval in 2019. These indications span multiple therapeutic areas including oncology, immunology, and more.

  • Hereditary Tyrosinemia Type 1
Source: FDA Label

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1 shared
CYCLE
Shared indications:
Hereditary Tyrosinemia Type 1
ORFADIN
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1 shared
SWEDISH ORPHAN
Shared indications:
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Clinical Trial Registry

8 trials
Trial Sponsor ID Phase Status Title
NCT01390077 results posted WLN02 Ph 2, Ph 3 completed Nitisinone (NTBC) In Different Age Groups Of Patients With Alkaptonuria
NCT01838655 results posted 130124 13-EI-0124 Ph 1, Ph 2 completed Nitisinone for Type 1B Oculocutaneous Albinism
NCT02750345 results posted CT-003 PXL227430 Ph 1 completed Bioequivalence Study of Two Oral Nitisinone Formulations to Treat Hereditary Tyrosinemia (HT-1)
NCT02750709 results posted CT-001 PXL225418 Ph 1 completed Bioequivalence Study of Two Nitisinone Formulations Compared to Orfadin
NCT02750332 results posted CT-002 PXL225421 Ph 1 completed Bioavailability Food-Effect Study of an Oral Nitisinone Formulation to Treat Hereditary Tyrosinemia (HT-1)
NCT02323529 HT-1 Sobi.NTBC-003 Ph 3 completed Efficacy and Safety of Once Daily Dosing Compared to Twice Daily Dosing of Nitisinone in HT-1
NCT01734889 results posted Sobi.NTBC-002 Ph 1 completed Taste and Palatability of Orfadin Suspension
NCT01828463 SONIA1 UoL000928 Ph 2 completed Dose Response Study of Nitisinone in Alkaptonuria
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Active Pipeline

Pro

Ongoing clinical trials by development phase

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Key Completed Trials

Pro

Completed studies with published results, ranked by significance

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Trial Timeline

Full development history with FDA approval milestones

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Understanding FDA Approval Types
Count Type What it means
- ORIG Original approval - drug first enters market
- SUPPL - Efficacy New indication (new disease/condition approved)
- SUPPL - Labeling Label text changes (warnings, dosing updates)
- SUPPL - Manufacturing Production changes (new facility)
- SUPPL - Chemistry Formulation changes (new dosage strength)

Green lines in the timeline show ORIG and Efficacy approvals - the clinically meaningful milestones.

NITISINONE FDA Label Details

Indications & Usage

NITISINONE is indicated for the treatment of Hereditary Tyrosinemia Type 1.

Looking for the branded version?

ORFADIN

Full clinical data, patents, trials, and competitive landscape for nitisinone.

See ORFADIN

Data Sources

Data sourced from official FDA and NIH databases. Click links to verify on original sources.