Skip to main content

Mitochondrial Diseases

10
Pipeline Programs
8
Companies
13
Clinical Trials
2 recruiting
0
Approved Products

Pipeline by Development Stage

Preclinical
Phase 1
Phase 1/2
Phase 2
Phase 2/3
Phase 3
On Market
0
1
1
7
0
1
0
Early DiscoveryClinical DevelopmentMarket

Drug Modality Breakdown

Peptide
2100%
+ 12 programs with unclassified modality

Competitive Landscape

8 companies ranked by most advanced pipeline stage

Khondrion
KhondrionNetherlands - Nijmegen
5 programs
4
1
SonlicromanolPhase 31 trial
KH176Phase 21 trial
KH176Phase 21 trial
Oral administration of 100 mg KH176 twice dailyPhase 21 trial
SonlicromanolPhase 21 trial
Active Trials
NCT04165239Completed27Est. May 2022
NCT02909400Completed20Est. Jul 2017
NCT04604548Completed11Est. Jun 2023
+2 more trials
Minovia Therapeutics
Minovia TherapeuticsIsrael - Tirat Hacarmel
2 programs
1
1
MNV-201Phase 21 trial
CD34+ cells enriched with MNV-BLDPhase 1/21 trial
Active Trials
NCT03384420Completed7Est. Mar 2021
NCT06017869Recruiting6Est. Dec 2027
Amgen
AmgenTHOUSAND OAKS, CA
1 program
1
Cysteamine BitartratePhase 21 trial
Active Trials
NCT02473445Terminated22Est. Mar 2017
Neovii Biotech
Neovii BiotechGermany - Grafelfing
1 program
1
EE-TPPhase 21 trial
Active Trials
NCT03866954Withdrawn0Est. Dec 2027
Repligen
RepligenWALTHAM, MA
1 program
1
RG2133Phase 11 trial
Active Trials
NCT00060515Terminated12
PTC Therapeutics
2 programs
EPI-743N/A1 trial
VatiquinonePHASE_2_31 trial
Active Trials
NCT01370447No Longer Available
NCT04378075Terminated68Est. Dec 2023
Stealth BioTherapeutics
1 program
elamipretideN/APeptide
BioTherapeutics Inc
BioTherapeutics IncVA - Blacksburg
1 program
elamipretideN/APeptide1 trial
Active Trials
NCT04689360Available

Trial Timeline

Clinical trial activity over time

2021
2022
2023
2024
2025
2026
2027
2028
KhondrionSonlicromanol
PTC TherapeuticsVatiquinone
Neovii BiotechEE-TP
Minovia TherapeuticsMNV-201
KhondrionOral administration of 100 mg KH176 twice daily
KhondrionSonlicromanol
KhondrionKH176
KhondrionKH176
AmgenCysteamine Bitartrate
Minovia TherapeuticsCD34+ cells enriched with MNV-BLD

Clinical Trials (13)

Total enrollment: 417 patients across 13 trials

KHENERFIN Study: A Trial to Evaluate the Efficacy and Safety of Sonlicromanol in Primary Mitochondrial Diseases

Start: Feb 2026Est. completion: Sep 2028220 patients
Phase 3Recruiting

A Study to Evaluate Efficacy and Safety of Vatiquinone for Treating Mitochondrial Disease in Participants With Refractory Epilepsy

Start: Sep 2020Est. completion: Dec 202368 patients
Phase 2/3Terminated

Trial of Erythrocyte Encapsulated Thymidine Phosphorylase In Mitochondrial Neurogastrointestinal Encephalomyopathy

Start: Nov 2024Est. completion: Dec 20270
Phase 2Withdrawn

Evaluate the Safety and Therapeutic Effects of a Single Intravenous Infusion (IV) of Autologous CD34+ Cells Enriched With Allogenic Placenta-derived Mitochondria in Patients With a Diagnosis of Pearson Syndrome (PS)

Start: Jul 2023Est. completion: Dec 20276 patients
Phase 2Recruiting
NCT04604548KhondrionOral administration of 100 mg KH176 twice daily

The KHENEREXT Study

Start: Aug 2021Est. completion: Jun 202311 patients
Phase 2Completed

The KHENERGYC Study

Start: Feb 2021Est. completion: Dec 202524 patients
Phase 2Suspended

The KHENERGYZE Study

Start: Oct 2019Est. completion: May 202227 patients
Phase 2Completed

The KHENERGY Study

Start: Sep 2016Est. completion: Jul 201720 patients
Phase 2Completed
NCT02473445AmgenCysteamine Bitartrate

A Long-term Extension of Study RP103-MITO-001 (NCT02023866) to Assess Cysteamine Bitartrate Delayed-release Capsules (RP103) in Children With Inherited Mitochondrial Disease

Start: May 2015Est. completion: Mar 201722 patients
Phase 2Terminated
NCT03384420Minovia TherapeuticsCD34+ cells enriched with MNV-BLD

A Study to Evaluate the Safety and Therapeutic Effects of Transplantation of MNV-BM-BLD in Pediatric Patients With Pearson Syndrome

Start: Feb 2019Est. completion: Mar 20217 patients
Phase 1/2Completed

RG2133 (2',3',5'-Tri-O-Acetyluridine) in Mitochondrial Disease

12 patients
Phase 1Terminated

An Intermediate Size Expanded Access Protocol of Elamipretide

N/AAvailable

EPI-743 for Mitochondrial Respiratory Chain Diseases

N/ANo Longer Available

Phase Legend

PreclinicalLab & animal studies
Phase 1Safety & dosing
Phase 2Efficacy testing
Phase 3Large-scale trials
On MarketApproved & available

Key Insights

1 late-stage (Phase 3) programs, potential near-term approvals
2 actively recruiting trials targeting 417 patients
8 companies competing in this space

The information on this page is for informational purposes only and should not be used as a substitute for professional medical advice. Drug information is sourced from FDA, DailyMed, and other government databases. Adverse event data from FAERS does not establish causation. Always consult a healthcare professional for medical decisions.