Werner J H Koopman Selected Research

Leigh Disease (Leigh's Disease)

1/2022	The decylTPP mitochondria-targeting moiety lowers electron transport chain supercomplex levels in primary human skin fibroblasts.
1/2022	Ndufs4 knockout mouse models of Leigh syndrome: pathophysiology and intervention.
11/2018	Rescue from galactose-induced death of Leigh Syndrome patient cells by pyruvate and NAD.
1/2015	Quantifying small molecule phenotypic effects using mitochondrial morpho-functional fingerprinting and machine learning.
3/2014	A guide to diagnosis and treatment of Leigh syndrome.
1/2013	OXPHOS mutations and neurodegeneration.
7/2008	Mitigation of NADH: ubiquinone oxidoreductase deficiency by chronic Trolox treatment.

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Werner J H Koopman Research Topics

Disease

11	Mitochondrial Diseases (Mitochondrial Disease) 01/2021 - 10/2009
7	Leigh Disease (Leigh's Disease) 01/2022 - 07/2008
4	Neoplasms (Cancer) 04/2019 - 10/2015
3	Mitochondrial complex I deficiency 01/2022 - 04/2011
2	Neurodegenerative Diseases (Neurodegenerative Disease) 01/2013 - 10/2005
1	Metabolic Syndrome (Dysmetabolic Syndrome X) 01/2021
1	Atherosclerosis 01/2021
1	Glioblastoma (Glioblastoma Multiforme) 04/2019
1	Carcinogenesis 01/2019
1	Acidosis 01/2018
1	Inflammation (Inflammations) 01/2018
1	Ischemia 01/2018
1	Ataxia Telangiectasia (Louis Bar Syndrome) 12/2016
1	Respiratory Insufficiency (Respiratory Failure) 01/2016
1	Muscular Diseases (Myopathy) 09/2015
1	Laminopathies 01/2015
1	Ataxia (Dyssynergia) 02/2013
1	Muscle Hypotonia (Hypotonia) 02/2013
1	Parkinson Disease (Parkinson's Disease) 04/2011
1	Cardiomyopathies (Cardiomyopathy) 07/2008
1	Brain Diseases (Brain Disorder) 10/2005

Drug/Important Bio-Agent (IBA)

5	Adenosine Triphosphate (ATP)IBA 01/2021 - 12/2009
4	Electron Transport Complex I (NADH-CoQ Reductase)IBA 01/2022 - 10/2005
3	AntioxidantsIBA 01/2022 - 12/2012
3	Mitochondrial DNA (mtDNA)IBA 01/2020 - 01/2013
2	DNA (Deoxyribonucleic Acid)IBA 01/2022 - 04/2011
2	6- hydroxy- 2,5,7,8- tetramethylchroman- 2- carboxylic acid (Trolox)IBA 01/2022 - 01/2015
2	Reactive Oxygen Species (Oxygen Radicals)IBA 12/2016 - 01/2015
2	EnzymesIBA 09/2015 - 10/2009
1	Vitamin E FDA LinkGeneric 01/2022
1	oxidized low density lipoproteinIBA 01/2021
1	6-chloro-2-(1-piperazinyl)pyrazine (CPP)IBA 04/2019
1	Elastin-Like PolypeptidesIBA 04/2019
1	Arginine (L-Arginine)FDA Link 04/2019
1	Dihydroorotate DehydrogenaseIBA 01/2019
1	Mitochondrial Proton-Translocating ATPases (Mitochondrial ATP Synthase)IBA 01/2019
1	pyrimidineIBA 01/2019
1	NAD (NADH)IBA 11/2018
1	Galactose (Galactopyranose)FDA LinkGeneric 11/2018
1	Pyruvic Acid (Pyruvate)IBA 11/2018
1	1- Methyl- 4- phenyl- 1,2,3,6- tetrahydropyridine (MPTP)IBA 01/2018
1	AMP-Activated Protein Kinase KinasesIBA 12/2016
1	Glucose (Dextrose)FDA LinkGeneric 12/2016
1	Antimycin AIBA 12/2016
1	piericidin AIBA 12/2016
1	Sirtuin 2IBA 12/2016
1	Isoflurane FDA LinkGeneric 01/2016
1	Electron Transport Complex III (Coenzyme Q-Cytochrome-c Reductase)IBA 09/2015
1	Hydroxymethylglutaryl-CoA Reductase Inhibitors (HMG-CoA Reductase Inhibitors)IBA 09/2015
1	Lamin Type A (Lamin A)IBA 01/2015
1	LaminsIBA 01/2015
1	prelamin AIBA 01/2015
1	Pyruvate Dehydrogenase Complex (Dehydrogenase Complex, Pyruvate)IBA 03/2014
1	coenzyme Q10 (CoQ10)IBA 03/2014
1	Electron Transport Complex IV (Cytochrome c Oxidase)IBA 02/2013
1	Pharmaceutical PreparationsIBA 12/2011
1	oligomycin sensitivity-conferring protein (complex V)IBA 11/2011
1	PTEN-induced putative kinaseIBA 04/2011

Therapy/Procedure

1	Ketogenic Diet 01/2021
1	Therapeutics 01/2015