| 1 |
Multiplexed high-content analysis of mitochondrial morphofunction using live-cell microscopy
Eligio F. Iannetti, Jan A.M. Smeitink, Julien Beyrath et al. · Nature Protocols
Open access |
18 Aug 2016 |
100 |
| 2 |
Mitochondrial disorders in children: toward development of small‐molecule treatment strategies
Werner J.H. Koopman, Julien Beyrath, Cheuk‐Wing Fung et al. · EMBO Molecular Medicine
Open access |
7 Mar 2016 |
92 |
| 3 |
Inducing cancer indolence by targeting mitochondrial Complex I is potentiated by blocking macrophage-mediated adaptive responses
Ivana Kurelac et al. · Nature Communications
Open access |
22 Feb 2019 |
81 |
| 4 |
KH176 Safeguards Mitochondrial Diseased Cells from Redox Stress-Induced Cell Death by Interacting with the Thioredoxin System/Peroxiredoxin Enzyme Machinery
Julien Beyrath, Mina Pellegrini, G. Herma Renkema et al. · Scientific Reports
Open access |
20 Apr 2018 |
75 |
| 5 |
The KHENERGY Study: Safety and Efficacy of KH 176 in Mitochondrial m.3243A>G Spectrum Disorders
Mirian C. H. Janssen, Saskia Koene, Paul de Laat et al. · Clinical Pharmacology & Therapeutics
Open access |
30 Jul 2018 |
58 |
| 6 |
The histone methyltransferase G9a regulates tolerance to oxidative stress–induced energy consumption
Human Riahi, Carlijn Brekelmans, Sarah Foriel et al. · PLoS Biology
Open access |
12 Mar 2019 |
54 |
| 7 |
Therapeutic effects of the mitochondrial ROS-redox modulator KH176 in a mammalian model of Leigh Disease
Ria de Haas, Devashish Das, Alejandro Garanto et al. · Scientific Reports
Open access |
11 Sep 2017 |
50 |
| 8 |
SK channel-mediated metabolic escape to glycolysis inhibits ferroptosis and supports stress resistance in C. elegans
Inge E. Krabbendam, Birgit Honrath, Benjamin Dilberger et al. · Cell Death and Disease
Open access |
23 Apr 2020 |
48 |
| 9 |
KH176 under development for rare mitochondrial disease: a first in man randomized controlled clinical trial in healthy male volunteers
Saskia Koene, Edwin Spaans, Luc Van Bortel et al. · Orphanet Journal of Rare Diseases
Open access |
16 Oct 2017 |
48 |
| 10 |
Rescue from galactose-induced death of Leigh Syndrome patient cells by pyruvate and NAD+
Eligio F. Iannetti, Jan A.M. Smeitink, Peter H.G.M. Willems et al. · Cell Death and Disease
Open access |
14 Nov 2018 |
46 |
| 11 |
Therapy-Induced Senescence Enhances the Efficacy of HER2-Targeted Antibody–Drug Conjugates in Breast Cancer
Santiago Duro‐Sánchez, Mercedes Nadal‐Serrano, Marta Lalinde-Gutiérrez et al. · Cancer Research
Open access |
12 Oct 2022 |
45 |
| 12 |
Quantifying small molecule phenotypic effects using mitochondrial morpho-functional fingerprinting and machine learning
Lionel Blanchet, Jan A.M. Smeitink, Sjenet E. van Emst - de Vries et al. · Scientific Reports
Open access |
26 Jan 2015 |
43 |
| 13 |
Hypothesis: mPGES-1-Derived Prostaglandin E2, a So Far Missing Link in COVID-19 Pathophysiology?
Jan A.M. Smeitink, Xiaolan Jiang, S. A. Pecheritsyna et al. · Preprints.org
PreprintOpen access |
12 Apr 2020 |
36 |
| 14 |
To be or not to be pink(1): contradictory findings in an animal model for Parkinson’s disease
Ria de Haas, Lisa C M W Heltzel, Denise Tax et al. · Brain Communications
Open access |
2019 |
35 |
| 15 |
Toward high-content screening of mitochondrial morphology and membrane potential in living cells
Eligio F. Iannetti, Peter H.G.M. Willems, Mina Pellegrini et al. · The International Journal of Biochemistry & Cell Biology
Open access |
8 Feb 2015 |
35 |
| 16 |
Process analytical technique ( PAT ) miniaturization for monoclonal antibody aggregate detection in continuous downstream processing
Mariana N. São Pedro, Marieke E. Klijn, Michel H. M. Eppink et al. · Journal of Chemical Technology & Biotechnology
Open access |
15 Sep 2021 |
34 |
| 17 |
Oxidative switch drives mitophagy defects in dopaminergic parkin mutant patient neurons
Aurélie Schwartzentruber, Camilla Boschian, Fernanda Martins Lopes et al. · Scientific Reports
Open access |
23 Sep 2020 |
32 |
| 18 |
Dissecting Drug-Induced Cytotoxicity and Metabolic Dysfunction in Conditionally Immortalized Human Proximal Tubule Cells
Charlotte A. Hoogstraten, Jan A.M. Smeitink, Frans G. M. Rüssel et al. · Frontiers in Toxicology
Open access |
28 Feb 2022 |
30 |
| 19 |
Sonlicromanol improves neuronal network dysfunction and transcriptome changes linked to m.3243A>G heteroplasmy in iPSC-derived neurons
Teun M. Klein Gunnewiek, Anouk H. A. Verboven, Iris Pelgrim et al. · Stem Cell Reports
Open access |
29 Jul 2021 |
29 |
| 20 |
Mechanism of action and potential applications of selective inhibition of microsomal prostaglandin E synthase-1-mediated PGE2 biosynthesis by sonlicromanol’s metabolite KH176m
Xiaoying Jiang, G. Herma Renkema, Bas Pennings et al. · Scientific Reports
Open access |
13 Jan 2021 |
29 |
| 21 |
Mitochondrial diseases: Drosophila melanogaster as a model to evaluate potential therapeutics
Sarah Foriel, Peter H.G.M. Willems, Jan A.M. Smeitink et al. · The International Journal of Biochemistry & Cell Biology
|
7 Feb 2015 |
26 |
| 22 |
Live-Imaging Readouts and Cell Models for Phenotypic Profiling of Mitochondrial Function
Eligio F. Iannetti, Alessandro Prigione, Jan A.M. Smeitink et al. · Frontiers in Genetics
Open access |
28 Feb 2019 |
23 |
| 23 |
Natural 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
Karlijn Bouman et al. · BMC Neurology
Open access |
12 Aug 2021 |
22 |
| 24 |
Mitochondrial Migraine: Disentangling the angiopathy paradigm in m.3243A>G patients
Jan A.M. Smeitink, Saskia Koene, Julien Beyrath et al. · JIMD Reports
Open access |
Mar 2019 |
21 |
| 25 |
Feeding difficulties, a key feature of the Drosophila NDUFS4 mitochondrial disease model
Sarah Foriel, Julien Beyrath, Ilse Eidhof et al. · Disease Models & Mechanisms
Open access |
2018 |
20 |
| 26 |
Time to Change: A Systems Pharmacology Approach to Disentangle Mechanisms of Drug-Induced Mitochondrial Toxicity
Charlotte A. Hoogstraten, Jonathan J. Lyon, Jan A.M. Smeitink et al. · Pharmacological Reviews
Open access |
10 Jan 2023 |
18 |
| 27 |
Cognitive functioning and mental health in mitochondrial disease: A systematic scoping review
Inge‐Lot Klein, Kim F. E. van de Loo, Jan A.M. Smeitink et al. · Neuroscience & Biobehavioral Reviews
Open access |
11 Feb 2021 |
18 |
| 28 |
A Drosophila Mitochondrial Complex I Deficiency Phenotype Array
Sarah Foriel, G. Herma Renkema, Yvonne Lasarzewski et al. · Frontiers in Genetics
Open access |
27 Mar 2019 |
18 |
| 29 |
The Redox Modulating Sonlicromanol Active Metabolite KH176m and the Antioxidant MPG Protect Against Short-Duration Cardiac Ischemia-Reperfusion Injury
Yang Xiao, Karen Yim, Hong Zhang et al. · Cardiovascular Drugs and Therapy
Open access |
29 Apr 2021 |
17 |
| 30 |
Phase 2b program with sonlicromanol in patients with mitochondrial disease due to m.3243A>G mutation
Jan A.M. Smeitink, Just van Es, Brigitte Bosman et al. · Brain
Open access |
28 Aug 2024 |
16 |
| 31 |
A 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”)
Jan A.M. Smeitink, Rob van Maanen, Lonneke de Boer et al. · BMC Neurology
Open access |
27 Apr 2022 |
14 |
| 32 |
Synthetic ligands of death receptor 5 display a cell-selective agonistic effect at different oligomerization levels
Julien Beyrath, Neïla Chekkat, Cristian Roberto Smulski et al. · Oncotarget
Open access |
9 Jul 2016 |
14 |
| 33 |
Real‐time detection of mAb aggregates in an integrated downstream process
Mariana N. São Pedro, Madelène Isaksson, Joaquín Gomis‐Fons et al. · Biotechnology and Bioengineering
Open access |
13 Jun 2023 |
13 |
| 34 |
Metabolic impact of genetic and chemical ADP/ATP carrier inhibition in renal proximal tubule epithelial cells
Charlotte A. Hoogstraten, Maaike M. E. Jacobs, Guido de Boer et al. · Archives of Toxicology
Open access |
8 May 2023 |
9 |
| 35 |
Natural variability of daily physical activity measured by accelerometry in children with a mitochondrial disease
Pascal R. de Vries, Mariska M. H. P. Janssen, Edwin Spaans et al. · Mitochondrion
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20 Apr 2019 |
9 |
| 36 |
Design of a microfluidic mixer channel: First steps into creating a fluorescent dye‐based biosensor for mAb aggregate detection
Mariana N. São Pedro, Mafalda S. Santos, Michel H. M. Eppink et al. · Biotechnology Journal
Open access |
4 Nov 2022 |
8 |
| 37 |
Application of a fluorescent dye‐based microfluidic sensor for real‐time detection of mAb aggregates
Mariana N. São Pedro, Michel H. M. Eppink, Marcel Ottens · Biotechnology Progress
Open access |
10 May 2023 |
6 |
| 38 |
Sonlicromanol’s active metabolite KH176m normalizes prostate cancer stem cell mPGES-1 overexpression and inhibits cancer spheroid growth
Xiaolan Jiang, G. Herma Renkema, Jan A.M. Smeitink et al. · PLoS ONE
Open access |
9 Jul 2021 |
6 |
| 39 |
Sensitivity of primary mitochondrial disease fibroblasts to ferroptosis: The role of intracellular iron
S. A. Pecheritsyna, Melisa Emel Ermert, Emina Podhumljak et al. · Mitochondrion
Open access |
24 Jan 2026 |
3 |
| 40 |
Restoring cellular NAD(P)H levels by PPARα and LXRα stimulation to improve mitochondrial complex I deficiency
Sanne J.C.M. Frambach, Ria de Haas, Jan A.M. Smeitink et al. · Life Sciences
Open access |
22 Apr 2022 |
3 |
| 41 |
Community Consensus Guidelines to Support FAIR Data Standards in Clinical Research Studies in Primary Mitochondrial Disease
Amel Karaa, Laura E. MacMullen, John C. Campbell et al. · Advanced Genetics
Open access |
19 Dec 2021 |
3 |
| 42 |
A qualitative study assessing content validity of Neuro-QoL fatigue and PROMIS mental fatigue in m.3243A>G primary mitochondrial disease
Jan Smeitink, Charlotte A. Hoogstraten, Mirian C. H. Janssen et al. · Orphanet Journal of Rare Diseases
Open access |
26 Aug 2026 |
0 |
| 43 |
Digital Cognitive Testing in Mitochondrial Disease: Validity and Challenges for Clinical Trial Use
Oksana Pogoryelova, Jan Smeitink, Herma Renkema et al. · Annals of Clinical and Translational Neurology
Open access |
31 Jul 2026 |
0 |
| 44 |
The goal attainment scale in primary mitochondrial disease: Construct validity and lessons learned from a randomized controlled trial
Kristofoor E Leeuwenberg, Joanna IntHout, Jan T. Groothuis et al. · Molecular Genetics and Metabolism
Open access |
3 Mar 2026 |
0 |
| 45 |
Restoring oxidative phosphorylation enhances osteogenesis in mitochondrial DNA translation defective human bone marrow stromal cells
Paula Fernandez Guerra, Pernille Kirkegaard Kjær, Simone Karlsson Terp et al. · bioRxiv (Cold Spring Harbor Laboratory)
PreprintOpen access |
24 Dec 2024 |
0 |
| 46 |
Pyruvate dehydrogenase is a potential mitochondrial off-target for gentamicin based on in silico predictions and in vitro inhibition studies
Charlotte A. Hoogstraten, Jan B. Koenderink, Carolijn E. van Straaten et al. · Toxicology in Vitro
Open access |
28 Nov 2023 |
0 |
| 47 |
CONGENITAL MUSCULAR DYSTROPHIES
Karlijn Bouman, Jan T. Groothuis, Jonne Doorduin et al. · Neuromuscular Disorders
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18 Sep 2021 |
0 |
| 48 |
Exogenous NAD+ prevents galactose-induced death of Leigh Syndrome patient fibroblasts with isolated complex I deficiency
Werner J.H. Koopman, Eligio F. Iannetti, Jan A.M. Smeitink et al. · Biochimica et Biophysica Acta (BBA) - Bioenergetics
Open access |
Sep 2018 |
0 |