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Jan A M Smeitink Selected Research

Mitochondrial complex I deficiency

7/2022Restoring cellular NAD(P)H levels by PPARα and LXRα stimulation to improve mitochondrial complex I deficiency.
1/2021Stimulation of cholesterol biosynthesis in mitochondrial complex I-deficiency lowers reductive stress and improves motor function and survival in mice.
1/2015Quantifying small molecule phenotypic effects using mitochondrial morpho-functional fingerprinting and machine learning.
4/2011Mouse models for nuclear DNA-encoded mitochondrial complex I deficiency.

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Jan A M Smeitink Research Topics

Disease

24Mitochondrial Diseases (Mitochondrial Disease)
12/2021 - 11/2004
5Leigh Disease (Leigh's Disease)
01/2022 - 07/2008
5Muscular Diseases (Myopathy)
12/2021 - 07/2014
5MELAS Syndrome (Syndrome, MELAS)
12/2021 - 04/2008
4Mitochondrial complex I deficiency
07/2022 - 04/2011
4Lactic Acidosis
05/2015 - 07/2002
4Brain Diseases (Brain Disorder)
07/2014 - 10/2005
4Hypertrophic Cardiomyopathy (Hypertrophic Obstructive Cardiomyopathy)
10/2012 - 07/2002
3Mitochondrial Myopathies (Mitochondrial Myopathy)
08/2017 - 07/2002
2Disease Progression
07/2014 - 10/2012
2Muscle Hypotonia (Hypotonia)
02/2013 - 03/2007
2Neurodegenerative Diseases (Neurodegenerative Disease)
01/2013 - 10/2005
2Cardiomyopathies (Cardiomyopathy)
04/2011 - 07/2008
1Acute Kidney Injury (Acute Renal Failure)
01/2022
1merosin negative Muscular dystrophy congenital
08/2021
1Muscular Dystrophies (Muscular Dystrophy)
08/2021
1Ischemia
01/2021
1Necrosis
01/2021
1Reperfusion Injury
01/2021
1Cardiovascular Diseases (Cardiovascular Disease)
01/2020
1Atherosclerotic Plaque (Atheroma)
01/2020
1Diabetes Mellitus
12/2019
1Chronic Kidney Failure (Chronic Renal Failure)
12/2019
1Neoplasms (Cancer)
04/2019
1Glioblastoma (Glioblastoma Multiforme)
04/2019
1Parkinson Disease (Parkinson's Disease)
01/2019
1Ataxia Telangiectasia (Louis Bar Syndrome)
12/2016
1Obesity
09/2015
1Myotoxicity
09/2015
1Hearing Loss (Hearing Impairment)
03/2015
1Retinal Dystrophies
12/2013
1Atrophy
12/2013
1Type I 3-Methylglutaconic Aciduria
11/2013
1Mitochondrial encephalopathy
05/2013
1Coenzyme Q10 Deficiency
03/2013
1Ataxia (Dyssynergia)
02/2013
1Renal Tubular Acidosis (Distal Renal Tubular Acidosis)
10/2012
1Dystonia (Limb Dystonia)
06/2012
1Deafness (Deaf Mutism)
06/2012
1Infections
06/2012
1Stroke (Strokes)
09/2011

Drug/Important Bio-Agent (IBA)

9Mitochondrial DNA (mtDNA)IBA
12/2021 - 08/2006
6EnzymesIBA
09/2015 - 07/2002
5Proteins (Proteins, Gene)FDA Link
09/2009 - 07/2002
4AntioxidantsIBA
01/2022 - 12/2012
4Fatty Acids (Saturated Fatty Acids)IBA
12/2021 - 09/2009
4Electron Transport Complex I (NADH-CoQ Reductase)IBA
01/2018 - 10/2005
3CholesterolIBA
01/2021 - 06/2012
3Hydroxymethylglutaryl-CoA Reductase Inhibitors (HMG-CoA Reductase Inhibitors)IBA
01/2018 - 09/2015
2NAD (NADH)IBA
07/2022 - 11/2018
26- hydroxy- 2,5,7,8- tetramethylchroman- 2- carboxylic acid (Trolox)IBA
01/2022 - 01/2015
2Pharmaceutical PreparationsIBA
01/2022 - 12/2011
2CarbonIBA
12/2021 - 01/2019
2Glucose (Dextrose)FDA LinkGeneric
12/2021 - 12/2016
2Biological ProductsIBA
01/2021 - 02/2007
2Adenosine Triphosphate (ATP)IBA
11/2018 - 09/2015
2Pyruvic Acid (Pyruvate)IBA
11/2018 - 07/2002
2Reactive Oxygen Species (Oxygen Radicals)IBA
12/2016 - 01/2015
2Biomarkers (Surrogate Marker)IBA
01/2015 - 07/2014
2oligomycin sensitivity-conferring protein (complex V)IBA
05/2013 - 11/2011
2Electron Transport Complex IV (Cytochrome c Oxidase)IBA
02/2013 - 04/2011
1Vitamin EFDA LinkGeneric
01/2022
1CreatineIBA
12/2021
1Laminin (Merosin)IBA
08/2021
1Cytochromes c (Cytochrome c)IBA
01/2021
14-hydroxy-2-nonenal (4-hydroxynonenal)IBA
01/2021
1BezafibrateIBA
01/2020
1Peroxisome Proliferator-Activated Receptors (PPAR)IBA
01/2020
1Elastin-Like PolypeptidesIBA
04/2019
16-chloro-2-(1-piperazinyl)pyrazine (CPP)IBA
04/2019
1Arginine (L-Arginine)FDA Link
04/2019
1PTEN-induced putative kinaseIBA
01/2019
1Galactose (Galactopyranose)FDA LinkGeneric
11/2018
1Transfer RNA (tRNA)IBA
08/2017
1Antimycin AIBA
12/2016
1Sirtuin 2IBA
12/2016
1piericidin AIBA
12/2016
1AMP-Activated Protein Kinase KinasesIBA
12/2016
1Adenosine Diphosphate (ADP)IBA
09/2015
1Cannabinoid Receptors (Cannabinoid Receptor)IBA
09/2015
1ibipinabantIBA
09/2015
1Electron Transport Complex III (Coenzyme Q-Cytochrome-c Reductase)IBA
09/2015
1fibroblast growth factor 21IBA
07/2014
1Retinaldehyde (Retinal)IBA
12/2013
1Fluorescein (Funduscein)FDA LinkGeneric
12/2013
1Leucine (L-Leucine)FDA Link
11/2013
1TransferasesIBA
03/2013
1Creatine Kinase (Creatine Phosphokinase)IBA
10/2012
1PhosphatidylglycerolsIBA
06/2012
1Phospholipids (Phosphatides)FDA LinkGeneric
06/2012
1His Transfer RNAIBA
09/2011
1Ribosomal Proteins (Ribosomal Protein)IBA
04/2011
1DNA (Deoxyribonucleic Acid)IBA
04/2011
1DigitoninIBA
03/2010

Therapy/Procedure

4Therapeutics
01/2019 - 01/2015
1Kidney Transplantation
12/2019
1Transplantation
12/2019
1Renal Replacement Therapy (Therapies, Renal Replacement)
12/2019
1Complementary Therapies (Alternative Medicine)
05/2015
1Cochlear Implants (Cochlear Implant)
03/2015