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Gerbert Jansen

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Miranda D Stobbe; Antoine H C van Kampen; Sander M Houten; Gerbert Jansen; Perry D Moerland
Critical assessment of human metabolic pathway databases: a stepping stone for future integration.
A M Willemsen; Gerbert Jansen; J C Komen; S van Hooff; H R Waterham; P M T Brites; R J A Wanders; A H C van Kampen
Organization and integration of biomedical knowledge with concept maps for key peroxisomal pathways.
Gerbert Jansen; Ronald J A Wanders
Alpha-oxidation.

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All Publications

2011: Miranda D Stobbe; Antoine H C van Kampen; Sander M Houten; Gerbert Jansen; Perry D Moerland
Critical assessment of human metabolic pathway databases: a stepping stone for future integration.
BMC systems biology 2011;5():165.
2008: A M Willemsen; Gerbert Jansen; J C Komen; S van Hooff; H R Waterham; P M T Brites; R J A Wanders; A H C van Kampen
Organization and integration of biomedical knowledge with concept maps for key peroxisomal pathways.
Bioinformatics (Oxford, England) 2008;24(16):i21-7.
2006: Gerbert Jansen; Ronald J A Wanders
Alpha-oxidation.
Biochimica et biophysica acta 2006;1763(12):1403-12.
2005: Jolein Gloerich; Naomi van Vlies; Gerbert Jansen; Simone Denis; Jos P N Ruiter; MA van Werkhoven; Marinus Duran; Frédéric M Vaz; R J A Wanders; Sacha Ferdinandusse
A phytol-enriched diet induces changes in fatty acid metabolism in mice both via PPARalpha-dependent and -independent pathways.
Journal of lipid research 2005;46(4):716-26.
2004: Daan M van den Brink; Joram N I van Miert; Georges Dacremont; Jean-François Rontani; Gerbert Jansen; Ronald J A Wanders
Identification of fatty aldehyde dehydrogenase in the breakdown of phytol to phytanic acid.
Molecular genetics and metabolism 2004;82(1):33-7.
2004: Gerbert Jansen; Hans R Waterham; Ronald J A Wanders
Molecular basis of Refsum disease: sequence variations in phytanoyl-CoA hydroxylase (PHYH) and the PTS2 receptor (PEX7).
Human mutation 2004;23(3):209-18.
2003: Mridul Mukherji; Christopher J Schofield; Anthony S Wierzbicki; Gerbert Jansen; Ronald J A Wanders; Matthew D Lloyd
The chemical biology of branched-chain lipid metabolism.
Progress in lipid research 2003;42(5):359-76.
2003: Ronald J A Wanders; Gerbert Jansen; Matthew D Lloyd
Phytanic acid alpha-oxidation, new insights into an old problem: a review.
Biochimica et biophysica acta 2003;1631(2):119-35.
2003: Daan M van den Brink; Pedro Brites; Janet Haasjes; Anthony S Wierzbicki; John Mitchell; Michelle Lambert-Hamill; Jackie de Belleroche; Gerbert Jansen; Hans R Waterham; Ronald J A Wanders
Identification of PEX7 as the second gene involved in Refsum disease.
American journal of human genetics 2003;72(2):471-7.
2003: Ronald J A Wanders; Carlo W T van Roermund; Wouter Visser; Sacha Ferdinandusse; Gerbert Jansen; Daan M van den Brink; Jolein Gloerich; Hans R Waterham
Peroxisomal fatty acid alpha- and beta-oxidation in health and disease: new insights.
Advances in experimental medicine and biology 2003;544():293-302.
2003: Daan M van den Brink; Pedro Brites; Janet Haasjes; Anthony S Wierzbicki; John Mitchell; Michelle Lambert-Hamill; Jackie de Belleroche; Gerbert Jansen; Hans R Waterham; Ronald J A Wanders
Identification of PEX7 as the second gene involved in Refsum disease.
Advances in experimental medicine and biology 2003;544():69-70.
2001: Ronald J A Wanders; Gerbert Jansen; OH Skjeldal
Refsum disease, peroxisomes and phytanic acid oxidation: a review.
Journal of neuropathology and experimental neurology 2001;60(11):1021-31.
2001: Gerbert Jansen; Daan M van den Brink; Rob Ofman; O Draghici; Georges Dacremont; Ronald J A Wanders
Identification of pristanal dehydrogenase activity in peroxisomes: conclusive evidence that the complete phytanic acid alpha-oxidation pathway is localized in peroxisomes.
Biochemical and biophysical research communications 2001;283(3):674-9.
2001: Ronald J A Wanders; Peter Vreken; Sacha Ferdinandusse; Gerbert Jansen; Hans R Waterham; Carlo W T van Roermund; Elisabeth G van Grunsven
Peroxisomal fatty acid alpha- and beta-oxidation in humans: enzymology, peroxisomal metabolite transporters and peroxisomal diseases.
Biochemical Society transactions 2001;29(Pt 2):250-67.
2000: Anna W M Zomer; Bart van der Burg; Gerbert Jansen; R J Wanders; Bwee Tien Poll-The; Paul T van der Saag
Pristanic acid and phytanic acid: naturally occurring ligands for the nuclear receptor peroxisome proliferator-activated receptor alpha.
Journal of lipid research 2000;41(11):1801-7.
2000: Anna W M Zomer; Gerbert Jansen; Bart van der Burg; Nanda M Verhoeven; C Jakobs; Paul T van der Saag; R J Wanders; Bwee Tien Poll-The
Phytanoyl-CoA hydroxylase activity is induced by phytanic acid.
European journal of biochemistry / FEBS 2000;267(13):4063-7.
2000: Gerbert Jansen; Simone Denis; Nanda M Verhoeven; Cornelis Jakobs; Ronald J A Wanders
Phytanic acid alpha-oxidation in man: identification of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal enzyme with normal activity in Zellweger syndrome.
Journal of inherited metabolic disease 2000;23(4):421-4.
2000: Gerbert Jansen; Eveline M Hogenhout; Sacha Ferdinandusse; Hans R Waterham; Rob Ofman; Cornelis Jakobs; OH Skjeldal; Ronald J A Wanders
Human phytanoyl-CoA hydroxylase: resolution of the gene structure and the molecular basis of Refsum's disease.
Human molecular genetics 2000;9(8):1195-200.
2000: Ronald J A Wanders; Elisabeth G van Grunsven; Gerbert Jansen
Lipid metabolism in peroxisomes: enzymology, functions and dysfunctions of the fatty acid alpha- and beta-oxidation systems in humans.
Biochemical Society transactions 2000;28(2):141-9.
2000: MR Baumgartner; Gerbert Jansen; Nanda M Verhoeven; P A Mooyer; C Jakobs; Frank Roels; Marc Espeel; A Fourmaintraux; H Bellet; R J A Wanders; Jean-Marie Saudubray
Atypical refsum disease with pipecolic acidemia and abnormal catalase distribution.
Annals of neurology 2000;47(1):109-13.
1999: Gerbert Jansen; Rob Ofman; Simone Denis; Sacha Ferdinandusse; Eveline M Hogenhout; Cornelis Jakobs; Ronald J A Wanders
Phytanoyl-CoA hydroxylase from rat liver. Protein purification and cDNA cloning with implications for the subcellular localization of phytanic acid alpha-oxidation.
Journal of lipid research 1999;40(12):2244-54.
1999: Gerbert Jansen; Nanda M Verhoeven; Simone Denis; G Romeijn; Cornelis Jakobs; Herman J Ten Brink; Ronald J A Wanders
Phytanic acid alpha-oxidation: identification of 2-hydroxyphytanoyl-CoA lyase in rat liver and its localisation in peroxisomes.
Biochimica et biophysica acta 1999;1440(2-3):176-82.
1999: Gerbert Jansen; Sacha Ferdinandusse; Eveline M Hogenhout; Nanda M Verhoeven; Cornelis Jakobs; Ronald J A Wanders
Phytanoyl-CoA hydroxylase deficiency. Enzymological and molecular basis of classical Refsum disease.
Advances in experimental medicine and biology 1999;466():371-6.
1998: Gerbert Jansen; Sacha Ferdinandusse; OH Skjeldal; OH Stokke; C J de Groot; Cornelis Jakobs; Ronald J A Wanders
Molecular basis of Refsum disease: identification of new mutations in the phytanoyl-CoA hydroxylase cDNA.
Journal of inherited metabolic disease 1998;21(3):288-91.
1998: Gerbert Jansen; Stephanie J Mihalik; Paul A Watkins; Cornelis Jakobs; Hugo W Moser; Ronald J A Wanders
Characterization of phytanoyl-Coenzyme A hydroxylase in human liver and activity measurements in patients with peroxisomal disorders.
Clinica chimica acta; international journal of clinical chemistry 1998;271(2):203-11.
1997: Nanda M Verhoeven; RJ Wanders; D S M Schor; Gerbert Jansen; Cornelis Jakobs
Phytanic acid alpha-oxidation: decarboxylation of 2-hydroxyphytanoyl-CoA to pristanic acid in human liver.
Journal of lipid research 1997;38(10):2062-70.
1997: Gerbert Jansen; Rob Ofman; Sacha Ferdinandusse; Lodewijk IJlst; Anton O Muijsers; OH Skjeldal; OH Stokke; Cornelis Jakobs; G T N Besley; James Wraith; Ronald J A Wanders
Refsum disease is caused by mutations in the phytanoyl-CoA hydroxylase gene.
Nature genetics 1997;17(2):190-3.
1997: Vincent T Ramaekers; B Bosman; Gerbert Jansen; R J Wanders
Increased plasma malondialdehyde associated with cerebellar structural defects.
Archives of disease in childhood 1997;77(3):231-4.
1997: Ronald J A Wanders; L Vilarinho; H P Hartung; Georg F Hoffmann; P A Mooijer; Gerbert Jansen; Jan G M Huijmans; J B C de Klerk; Herman J Ten Brink; Cornelis Jakobs; Marinus Duran
L-2-Hydroxyglutaric aciduria: normal L-2-hydroxyglutarate dehydrogenase activity in liver from two new patients.
Journal of inherited metabolic disease 1997;20(5):725-6.
1997: Gerbert Jansen; Ronald J A Wanders; Paul A Watkins; Stephanie J Mihalik
Phytanoyl-coenzyme A hydroxylase deficiency -- the enzyme defect in Refsum's disease.
The New England journal of medicine 1997;337(2):133-4.
1997: Gerbert Jansen; Stephanie J Mihalik; Paul A Watkins; Hugo W Moser; Cornelis Jakobs; Hugo S A Heijmans; Ronald J A Wanders
Phytanoyl-CoA hydroxylase is not only deficient in classical Refsum disease but also in rhizomelic chondrodysplasia punctata.
Journal of inherited metabolic disease 1997;20(3):444-6.
1997: Nanda M Verhoeven; D S M Schor; Gerbert Jansen; R M Kok; Herman J Ten Brink; RJ Wanders; Cornelis Jakobs
Formation of 2,3-pristenic acid and 3-hydroxypristanic acid from pristanic acid in human liver.
Journal of inherited metabolic disease 1997;20(3):441-3.
1997: Gerbert Jansen; R J Wanders
Plasmalogens and oxidative stress: evidence against a major role of plasmalogens in protection against the superoxide anion radical.
Journal of inherited metabolic disease 1997;20(1):85-94.
1996: Gerbert Jansen; Stephanie J Mihalik; Paul A Watkins; Hugo W Moser; C Jakobs; Simone Denis; R J Wanders
Phytanoyl-CoA hydroxylase is present in human liver, located in peroxisomes, and deficient in Zellweger syndrome: direct, unequivocal evidence for the new, revised pathway of phytanic acid alpha-oxidation in humans.
Biochemical and biophysical research communications 1996;229(1):205-10.
1996: Gerbert Jansen; R J Wanders; G Joost Jöbsis; PA Bolhuis; J M de Jong
Evidence against increased oxidative stress in fibroblasts from patients with non-superoxide-dismutase-1 mutant familial amyotrophic lateral sclerosis.
Journal of the neurological sciences 1996;139 Suppl():91-4.
1993: Gerbert Jansen; R J Wanders
L-2-hydroxyglutarate dehydrogenase: identification of a novel enzyme activity in rat and human liver. Implications for L-2-hydroxyglutaric acidemia.
Biochimica et biophysica acta 1993;1225(1):53-6.
1993: P G Barth; G F Hoffmann; Jaak Jaeken; R J Wanders; Marinus Duran; Gerbert Jansen; C Jakobs; W Lehnert; F Hanefeld; J Valk
L-2-hydroxyglutaric acidaemia: clinical and biochemical findings in 12 patients and preliminary report on L-2-hydroxyacid dehydrogenase.
Journal of inherited metabolic disease 1993;16(4):753-61.