Ronald J A Wanders Selected Research

ATP-Binding Cassette Transporters (ABC Transporters)

1/2019	Mutagenesis separates ATPase and thioesterase activities of the peroxisomal ABC transporter, Comatose.
1/2013	Intrinsic acyl-CoA thioesterase activity of a peroxisomal ATP binding cassette transporter is required for transport and metabolism of fatty acids.
11/2012	Bezafibrate lowers very long-chain fatty acids in X-linked adrenoleukodystrophy fibroblasts by inhibiting fatty acid elongation.
12/2011	Mammalian peroxisomal ABC transporters: from endogenous substrates to pathology and clinical significance.
3/2011	Differential substrate specificities of human ABCD1 and ABCD2 in peroxisomal fatty acid β-oxidation.
9/2010	The Arabidopsis peroxisomal ABC transporter, comatose, complements the Saccharomyces cerevisiae pxa1 pxa2Delta mutant for metabolism of long-chain fatty acids and exhibits fatty acyl-CoA-stimulated ATPase activity.
3/2010	The role of ELOVL1 in very long-chain fatty acid homeostasis and X-linked adrenoleukodystrophy.
12/2005	Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage.

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Ronald J A Wanders Research Topics

Disease

31	Adrenoleukodystrophy (Adrenoleukodystrophy, X-Linked) 12/2017 - 04/2002
21	Peroxisomal Disorders (Peroxisomal Disorder) 11/2023 - 02/2003
18	Zellweger Syndrome (Zellweger's Syndrome) 01/2021 - 01/2002
15	Hypoglycemia (Reactive Hypoglycemia) 01/2022 - 01/2002
11	Inborn Genetic Diseases (Disease, Hereditary) 05/2024 - 03/2003
10	Barth Syndrome 01/2022 - 09/2002
10	Refsum Disease (Refsum's Disease) 12/2020 - 02/2003
9	Sjogren-Larsson Syndrome 01/2024 - 05/2004
9	Peroxisome biogenesis disorders 01/2021 - 03/2002
8	Neurodegenerative Diseases (Neurodegenerative Disease) 11/2023 - 02/2005
8	Muscular Diseases (Myopathy) 06/2021 - 11/2002
8	Cardiomyopathies (Cardiomyopathy) 01/2020 - 12/2003
7	VLCAD deficiency 01/2020 - 07/2004
6	Multiple Acyl Coenzyme A Dehydrogenase Deficiency 01/2024 - 12/2005
6	Mitochondrial Diseases (Mitochondrial Disease) 01/2021 - 09/2005
6	Inborn Errors Metabolism (Inborn Errors of Metabolism) 01/2021 - 06/2013
6	Liver Diseases (Liver Disease) 10/2019 - 12/2003
6	Disease Progression 01/2019 - 01/2002
6	Trifunctional Protein Deficiency With Myopathy And Neuropathy 01/2016 - 01/2002
6	Mevalonate Kinase Deficiency 07/2006 - 10/2002
5	Primary hyperoxaluria type 1 01/2024 - 08/2004
5	Intellectual Disability (Idiocy) 11/2022 - 06/2010
5	Brain Diseases (Brain Disorder) 01/2021 - 04/2004
5	Hyperammonemia 01/2019 - 01/2007
5	Seizures (Absence Seizure) 01/2018 - 08/2004
5	Insulin Resistance 01/2016 - 11/2008
5	Fever (Fevers) 07/2006 - 12/2002
4	Epilepsy (Aura) 01/2023 - 11/2004
4	Hepatomegaly 06/2021 - 04/2002
4	Rhabdomyolysis 06/2021 - 06/2005
4	Metabolic Diseases (Metabolic Disease) 01/2021 - 08/2006
4	Coma (Comas) 01/2019 - 09/2010
4	Rhizomelic Chondrodysplasia Punctata 08/2016 - 04/2002
4	Protein Deficiency 11/2012 - 06/2003
4	Medium chain acyl CoA dehydrogenase deficiency 05/2012 - 10/2002
4	Short chain Acyl CoA dehydrogenase deficiency 10/2010 - 11/2003
3	Hepatocellular Carcinoma (Hepatoma) 01/2024 - 03/2006
3	Nervous System Diseases (Neurological Disorders) 12/2021 - 10/2007
3	Ataxia (Dyssynergia) 11/2019 - 11/2008
3	Body Weight (Weight, Body) 01/2019 - 11/2008
3	Leukoencephalopathies 01/2017 - 11/2004
3	Beta-Hydroxyisobutyryl CoA Deacylase Deficiency 08/2015 - 01/2007
3	Ketosis 11/2014 - 11/2005
3	Neutropenia 09/2013 - 11/2002
3	Muscle Hypotonia (Hypotonia) 11/2012 - 08/2006
3	Syndromic 10 X-Linked Mental Retardation 02/2007 - 05/2002
3	Type I 3-Methylglutaconic Aciduria 04/2006 - 12/2002

Drug/Important Bio-Agent (IBA)

59	Fatty Acids (Saturated Fatty Acids)IBA 06/2021 - 01/2002
47	EnzymesIBA 05/2024 - 01/2002
25	Proteins (Proteins, Gene)FDA Link 01/2021 - 04/2002
13	Carnitine (L-Carnitine)FDA LinkGeneric 11/2020 - 06/2003
12	Oxidoreductases (Dehydrogenase)IBA 01/2022 - 06/2005
12	Phytanic AcidIBA 12/2020 - 02/2003
12	Coenzyme A (CoA)IBA 12/2018 - 11/2005
10	Cardiolipins (Cardiolipin)IBA 01/2022 - 09/2002
9	LipidsIBA 12/2021 - 12/2003
8	long-chain-aldehyde dehydrogenaseIBA 01/2024 - 05/2004
8	Bile Acids and Salts (Bile Acids)IBA 10/2019 - 01/2007
8	ATP-Binding Cassette Transporters (ABC Transporters)IBA 01/2019 - 12/2005
8	AcidsIBA 01/2015 - 11/2004
7	acylcarnitineIBA 01/2020 - 10/2002
6	Long-Chain Acyl-CoA Dehydrogenase (Long-Chain-Acyl-Coenzyme A Dehydrogenase)IBA 01/2020 - 07/2004
6	Acyl Coenzyme A (Acyl CoA)IBA 01/2019 - 08/2006
6	Phospholipids (Phosphatides)FDA LinkGeneric 01/2018 - 12/2003
6	Mitochondrial Trifunctional ProteinIBA 01/2013 - 06/2003
5	Amino Acids FDA Link 09/2021 - 06/2009
5	DNA (Deoxyribonucleic Acid)IBA 01/2019 - 11/2003
5	mevalonate kinaseIBA 07/2006 - 10/2002
4	glyoxylic acid (glyoxylate)IBA 01/2024 - 08/2004
4	Biomarkers (Surrogate Marker)IBA 01/2024 - 01/2003
4	Valine (L-Valine)FDA Link 01/2022 - 01/2007
4	type 1 Rhizomelic chondrodysplasia punctataIBA 01/2017 - 02/2003
4	BezafibrateIBA 01/2016 - 01/2012
4	Acyl-CoA Dehydrogenase (Medium-Chain Acyl-Coenzyme A Dehydrogenase)IBA 01/2013 - 10/2002
4	Fatty AlcoholsIBA 07/2012 - 07/2005
4	phytanoyl-coenzyme A (phytanoyl CoA)IBA 09/2011 - 02/2003
4	CholesterolIBA 05/2011 - 01/2002
4	Isoleucine (L-Isoleucine)FDA Link 02/2006 - 05/2002
3	VitaminsIBA 01/2023 - 01/2019
3	Galactose (Galactopyranose)FDA LinkGeneric 11/2022 - 05/2005
3	OxalatesIBA 12/2021 - 05/2005
3	Electron-Transferring Flavoproteins (Electron Transfer Flavoprotein)IBA 01/2021 - 12/2005
3	Flavin-Adenine Dinucleotide (FAD)IBA 01/2021 - 12/2005
3	Lactic Acid (Lactate)FDA LinkGeneric 01/2021 - 08/2010
3	KetonesIBA 01/2021 - 11/2014
3	Glucose (Dextrose)FDA LinkGeneric 09/2020 - 12/2013
3	Cholic AcidIBA 01/2019 - 11/2016
3	Unsaturated Fatty Acids (Polyunsaturated Fatty Acids)IBA 09/2014 - 01/2004
3	Mitochondrial DNA (mtDNA)IBA 05/2014 - 12/2003
3	Adenosine Triphosphate (ATP)IBA 01/2013 - 03/2011
3	Riboflavin (Vitamin B2)FDA LinkGeneric 10/2012 - 06/2009
3	PlasmalogensIBA 01/2011 - 10/2003
3	2- cyclohexylidenhydrazo- 4- phenyl- thiazoleIBA 03/2010 - 11/2005
3	Mixed Function Oxygenases (Monooxygenases)IBA 11/2008 - 02/2003
3	Terpenes (Terpenoids)IBA 07/2006 - 10/2002
3	PhytolIBA 07/2005 - 05/2004
3	Immunoglobulin D (IgD)IBA 02/2003 - 12/2002
2	Alanine-glyoxylate transaminase (alanine-glyoxylate aminotransferase)IBA 01/2024 - 05/2005
2	Mitogen-Activated Protein KinasesIBA 11/2023 - 12/2006

Therapy/Procedure

8	Therapeutics 01/2019 - 01/2002