Khondrion

mitochondrial disease, small molecule, sonlicromanol

modulatingsmall molecule

khondrion.com·Netherlands·

01Overview 02People 03Projects 04Concepts 05Patents 06Public records 07Belief evolution

Plate C · C-DQSC

Summary · Eternal Search editorial

Khondrion mitochondrial disease, small molecule, sonlicromanol

Khondrion is a clinical-stage biopharmaceutical company discovering and developing therapies targeting mitochondrial disease. Based on proprietary science and a deep understanding of mitochondrial dysfunction, it is advancing wholly owned lead drug candidate sonlicromanol for children and adults. The company is running clinical studies including the Phase 3 KHENERFIN study and a Phase II study targeting Post-COVID fatigue.

The published score reflects a curated read of public evidence across 16 dimensions. Each dimension below shows the rationale we used. Disagree with a reading? Tell us where we are wrong →

Plate C·01 · Scientific profile

All 16 dimensions
on the record.

Composite14

Chain scale100 pts

Dimensions16

Plate C·02 · Where it sits among peers

Score vs. proximity
to longevity goals.

Higher up = higher Eternal Search score. Further right = closer to achieving real longevity outcomes (early mechanism → translational → near-term readout). Bubble size = capital deployed. Watch for MISMATCHES: big bubbles on the left are well-capitalized but far from goals; small bubbles on the right are doing more with less.

Reading

Khondrion — Near-term readout · Bottom quartile. Derived deterministically from 5 extracted project(s). Most-advanced status: phase 3. Band: near_readout. Aging-relevance not LLM-reviewed in this pass.

Source: Deterministic

At a glance

Webkhondrion.com

HQNetherlands

Publications

29 on file

012026The goal attainment scale in primary mitochondrial disease: Construct validity and lessons learned from a randomized controlled trial.Molecular genetics and metabolism→022026Sensitivity of primary mitochondrial disease fibroblasts to ferroptosis: The role of intracellular iron.Mitochondrion→032025Phase 2b program with sonlicromanol in patients with mitochondrial disease due to m.3243A>G mutation.Brain : a journal of neurology→042024Pyruvate dehydrogenase is a potential mitochondrial off-target for gentamicin based on in silico predictions and in vitro inhibition studies.Toxicology in vitro : an international journal published in association with BIBRA→052023Time to Change: A Systems Pharmacology Approach to Disentangle Mechanisms of Drug-Induced Mitochondrial Toxicity.Pharmacological reviews→062022Restoring cellular NAD(P)H levels by PPARα and LXRα stimulation to improve mitochondrial complex I deficiency.Life sciences→072022A randomised placebo-controlled, double-blind phase II study to explore the safety, efficacy, and pharmacokinetics of sonlicromanol in children with genetically confirmed mitochondrial disease and motor symptoms ("KHENERGYC").BMC neurology→082021Sonlicromanol improves neuronal network dysfunction and transcriptome changes linked to m.3243A>G heteroplasmy in iPSC-derived neurons.Stem cell reports→092021The Redox Modulating Sonlicromanol Active Metabolite KH176m and the Antioxidant MPG Protect Against Short-Duration Cardiac Ischemia-Reperfusion Injury.Cardiovascular drugs and therapy→102021Natural history, outcome measures and trial readiness in LAMA2-related muscular dystrophy and SELENON-related myopathy in children and adults: protocol of the LAST STRONG study.BMC neurology→112021Sonlicromanol's active metabolite KH176m normalizes prostate cancer stem cell mPGES-1 overexpression and inhibits cancer spheroid growth.PloS one→122021Cognitive functioning and mental health in mitochondrial disease: A systematic scoping review.Neuroscience and biobehavioral reviews→132021Mechanism of action and potential applications of selective inhibition of microsomal prostaglandin E synthase-1-mediated PGE2 biosynthesis by sonlicromanol's metabolite KH176m.Scientific reports→142020Oxidative switch drives mitophagy defects in dopaminergic parkin mutant patient neurons.Scientific reports→152019A Drosophila Mitochondrial Complex I Deficiency Phenotype Array.Frontiers in genetics→162019Mitochondrial Migraine: Disentangling the angiopathy paradigm in m.3243A>G patients.JIMD reports→172019Live-Imaging Readouts and Cell Models for Phenotypic Profiling of Mitochondrial Function.Frontiers in genetics→182019The KHENERGY Study: Safety and Efficacy of KH176 in Mitochondrial m.3243A>G Spectrum Disorders.Clinical pharmacology and therapeutics→192019Natural variability of daily physical activity measured by accelerometry in children with a mitochondrial disease.Mitochondrion→202018KH176 Safeguards Mitochondrial Diseased Cells from Redox Stress-Induced Cell Death by Interacting with the Thioredoxin System/Peroxiredoxin Enzyme Machinery.Scientific reports→212018Feeding difficulties, a key feature of the Drosophila NDUFS4 mitochondrial disease model.Disease models & mechanisms→222018Rescue from galactose-induced death of Leigh Syndrome patient cells by pyruvate and NAD.Cell death & disease→232017KH176 under development for rare mitochondrial disease: a first in man randomized controlled clinical trial in healthy male volunteers.Orphanet journal of rare diseases→242017Therapeutic effects of the mitochondrial ROS-redox modulator KH176 in a mammalian model of Leigh Disease.Scientific reports→252016Multiplexed high-content analysis of mitochondrial morphofunction using live-cell microscopy.Nature protocols→262016Mitochondrial disorders in children: toward development of small-molecule treatment strategies.EMBO molecular medicine→272015Mitochondrial diseases: Drosophila melanogaster as a model to evaluate potential therapeutics.The international journal of biochemistry & cell biology→282015Quantifying small molecule phenotypic effects using mitochondrial morpho-functional fingerprinting and machine learning.Scientific reports→292015Toward high-content screening of mitochondrial morphology and membrane potential in living cells.The international journal of biochemistry & cell biology→

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0-100 chain-logic scale · 15 dimensions · scored on public evidence

Khondrion

All 16 dimensionson the record.

Score vs. proximityto longevity goals.

Publications

Loading

All 16 dimensionson the record.

Score vs. proximityto longevity goals.

Publications

All 16 dimensions
on the record.

Score vs. proximity
to longevity goals.

All 16 dimensions
on the record.

Score vs. proximity
to longevity goals.