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KH176 by Khondrion for m.3243A>G mitochondrial disease

Also known as: KH176, Sonlicromanol, KHENEREXT, KH176-203, NCT04604548

Khondrion BV

Primary source

Company

Khondrion BV

Program Type

Therapeutic

Modality

Small Molecule

Stage

Phase 2

Status

Completed

Target Gene

MT-TL1

Route / Mode

Oral tablet

Confidence

86%

Status Confidence

Status needs checking

Evidence Strength

Needs more support

Last Reviewed

2026-06-09

Geography

Denmark, Germany, Netherlands, United Kingdom

Population

adult, genetic, mitochondrial-disease, midd, hearing-endpoints

Trial IDs

Mechanism

Orally bioavailable redox-modulating small molecule evaluated for mitochondrial disease caused by the m.3243A>G mutation, including MIDD and hearing-related endpoints such as pure-tone and speech audiometry.

Indications

Hearing Loss Genetic Hearing Loss Mitochondrial Disease Maternally Inherited Diabetes and Deafness

Notes

Verified as a Khondrion-sponsored Phase 2 open-label extension of KH176 in m.3243A>G mitochondrial disease. It is relevant to HearScape through MIDD/deafness and explicit audiometry endpoints, but it is not a hearing-loss-only program.

Evidence & Sources

1 structured sources

clinicaltrials

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IDs: NCT04604548, KH176-203, KHENEREXT

Open

Key Events

2021-08-09

KHENEREXT Phase 2 extension started

2023-06-01

ClinicalTrials.gov lists the study as completed

Clinical Trials (1)