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Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation: A review

Journal of inherited metabolic disease, 2008-04, Vol.31 (2), p.205-216

2008

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Advances in methods for characterization of hepatic urea cycle enzymatic activity in HepaRG cells using UPLC-MS/MS

Analytical biochemistry, 2017-10, Vol.535, p.47-55

2017

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Inhibition of N-acetylglutamate synthase by various monocarboxylic and dicarboxylic short-chain coenzyme A esters and the production of alternative glutamate esters

Biochimica et biophysica acta, 2014-12, Vol.1842 (12), p.2510-2516

2014

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Clinical variability of isovaleric acidemia in a genetically homogeneous population

Journal of inherited metabolic disease, 2012-11, Vol.35 (6), p.1021-1029

2012

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Processing of mutant N-acetyl-α-glucosaminidase in mucopolysaccharidosis type IIIB fibroblasts cultured at low temperature

Molecular genetics and metabolism, 2017-09, Vol.122 (1-2), p.100-106

2017

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Fatty acid metabolism in Saccharomyces cerevisiae

Cellular and molecular life sciences : CMLS, 2003-09, Vol.60 (9), p.1838-1851

2003

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NAD depletion and mitochondrial protein hyperacetylation: New insights on liver toxicity and drug-induced effects in vivo

Toxicology letters, 2015-10, Vol.238 (2), p.S321-S321

2015

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Subcellular localization and physiological role of alpha-methylacyl-CoA racemase

Journal of lipid research, 2000-11, Vol.41 (11), p.1890-1896

2000

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Heparan sulfate derived disaccharides in plasma and total urinary excretion of glycosaminoglycans correlate with disease severity in Sanfilippo disease

Journal of inherited metabolic disease, 2013-03, Vol.36 (2), p.271-279

2013

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3-Methylglutaconic Aciduria Type I Is Caused by Mutations in AUH

American journal of human genetics, 2002-12, Vol.71 (6), p.1463-1466

2002

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Refsum disease is caused by mutations in the phytanoyl-CoA hydroxylase gene

Nature genetics, 1997-10, Vol.17 (2), p.190-193

1997

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Identification of a Peroxisomal ATP Carrier Required for Medium-Chain Fatty Acid β-Oxidation and Normal Peroxisome Proliferation in Saccharomyces cerevisiae

Molecular and Cellular Biology, 2001-07, Vol.21 (13), p.4321-4329

2001

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Disorders of mitochondrial fatty acyl‐CoA β‐oxidation

Journal of inherited metabolic disease, 1999-06, Vol.22 (4), p.442-487

1999

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Bezafibrate induces FALDH in human fibroblasts; implications for Sjögren-Larsson syndrome

Molecular genetics and metabolism, 2006-09, Vol.89 (1), p.111-115

2006

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Differential inhibitory effect of long-chain acyl-CoA esters on succinate and glutamate transport into rat liver mitochondria and its possible implications for long-chain fatty acid oxidation defects

Molecular genetics and metabolism, 2005-11, Vol.86 (3), p.344-352

2005

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Acyl-CoA dehydrogenase 9 (ACAD 9) is the long-chain acyl-CoA dehydrogenase in human embryonic and fetal brain

Biochemical and biophysical research communications, 2006-07, Vol.346 (1), p.33-37

2006

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Molecular basis of hepatic carnitine palmitoyltransferase I deficiency

The Journal of clinical investigation, 1998-08, Vol.102 (3), p.527-531

1998

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High prevalence of short-chain acyl-CoA dehydrogenase deficiency in the Netherlands, but no association with epilepsy of unknown origin in childhood

Neuropediatrics, 2011-02, Vol.42 (1), p.13-17

2011

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Functional Analysis of Mutant Human Carnitine Acylcarnitine Translocases in Yeast

Biochemical and biophysical research communications, 2001-01, Vol.280 (3), p.700-706

2001

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Reinvestigation of Peroxisomal 3-Ketoacyl-CoA Thiolase Deficiency: Identification of the True Defect at the Level of d-Bifunctional Protein

American journal of human genetics, 2002-06, Vol.70 (6), p.1589-1593

2002

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Ether lipid biosynthesis: alkyl-dihydroxyacetonephosphate synthase protein deficiency leads to reduced dihydroxyacetonephosphate acyltransferase activities

Journal of lipid research, 1999-11, Vol.40 (11), p.1998-2003

1999

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Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: clinical presentation and follow-up of 50 patients

Pediatrics (Evanston), 2002-01, Vol.109 (1), p.99-104

2002

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Identification of fatty acid oxidation disorder patients with lowered acyl-CoA thioesterase activity in human skin fibroblasts

European journal of clinical investigation, 2005-01, Vol.35 (1), p.38-46

2005

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A novel molecular defect of the carnitine acylcarnitine translocase gene in a Saudi patient

Clinical genetics, 2003-08, Vol.64 (2), p.163-165

2003

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Identification of the human mitochondrial FAD transporter and its potential role in multiple acyl-CoA dehydrogenase deficiency

Molecular genetics and metabolism, 2005-12, Vol.86 (4), p.441-447

2005

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Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation: A review

Advances in methods for characterization of hepatic urea cycle enzymatic activity in HepaRG cells using UPLC-MS/MS

Inhibition of N-acetylglutamate synthase by various monocarboxylic and dicarboxylic short-chain coenzyme A esters and the production of alternative glutamate esters

Clinical variability of isovaleric acidemia in a genetically homogeneous population

Processing of mutant N-acetyl-α-glucosaminidase in mucopolysaccharidosis type IIIB fibroblasts cultured at low temperature

Fatty acid metabolism in Saccharomyces cerevisiae

NAD depletion and mitochondrial protein hyperacetylation: New insights on liver toxicity and drug-induced effects in vivo

Subcellular localization and physiological role of alpha-methylacyl-CoA racemase

Heparan sulfate derived disaccharides in plasma and total urinary excretion of glycosaminoglycans correlate with disease severity in Sanfilippo disease

3-Methylglutaconic Aciduria Type I Is Caused by Mutations in AUH

Refsum disease is caused by mutations in the phytanoyl-CoA hydroxylase gene

Identification of a Peroxisomal ATP Carrier Required for Medium-Chain Fatty Acid β-Oxidation and Normal Peroxisome Proliferation in Saccharomyces cerevisiae

Disorders of mitochondrial fatty acyl‐CoA β‐oxidation

Bezafibrate induces FALDH in human fibroblasts; implications for Sjögren-Larsson syndrome

Differential inhibitory effect of long-chain acyl-CoA esters on succinate and glutamate transport into rat liver mitochondria and its possible implications for long-chain fatty acid oxidation defects

Acyl-CoA dehydrogenase 9 (ACAD 9) is the long-chain acyl-CoA dehydrogenase in human embryonic and fetal brain

Molecular basis of hepatic carnitine palmitoyltransferase I deficiency

High prevalence of short-chain acyl-CoA dehydrogenase deficiency in the Netherlands, but no association with epilepsy of unknown origin in childhood

Functional Analysis of Mutant Human Carnitine Acylcarnitine Translocases in Yeast

Reinvestigation of Peroxisomal 3-Ketoacyl-CoA Thiolase Deficiency: Identification of the True Defect at the Level of d-Bifunctional Protein

Ether lipid biosynthesis: alkyl-dihydroxyacetonephosphate synthase protein deficiency leads to reduced dihydroxyacetonephosphate acyltransferase activities

Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: clinical presentation and follow-up of 50 patients

Identification of fatty acid oxidation disorder patients with lowered acyl-CoA thioesterase activity in human skin fibroblasts

A novel molecular defect of the carnitine acylcarnitine translocase gene in a Saudi patient

Identification of the human mitochondrial FAD transporter and its potential role in multiple acyl-CoA dehydrogenase deficiency

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