Johanna E Groener

Summary

Affiliation: Academic Medical Center
Country: The Netherlands

Publications

  1. pmc Consequences of a global enzyme shortage of agalsidase beta in adult Dutch Fabry patients
    Bouwien E Smid
    Department of Internal Medicine, Division of Endocrinology and Metabolism, Academic Medical Centre, PO Box 22660, 1100 DD, Amsterdam, The Netherlands
    Orphanet J Rare Dis 6:69. 2011
  2. doi Ceramide in lipid emulsions used in parenteral nutrition: an innocent bystander?
    Johanna E Groener
    Department of Medical Biochemistry, Academic Medical Center, Amsterdam, The Netherlands
    JPEN J Parenter Enteral Nutr 35:270-1. 2011
  3. ncbi Plasma glucosylceramide and ceramide in type 1 Gaucher disease patients: correlations with disease severity and response to therapeutic intervention
    J E M Groener
    Department of Medical Biochemistry, University of Amsterdam, Academic Medical Center, Amsterdam, The Netherlands
    Biochim Biophys Acta 1781:72-8. 2008
  4. ncbi HPLC for simultaneous quantification of total ceramide, glucosylceramide, and ceramide trihexoside concentrations in plasma
    Johanna E M Groener
    Department of Medical Biochemistry, University of Amsterdam, Academic Medical Center, Amsterdam, The Netherlands
    Clin Chem 53:742-7. 2007
  5. ncbi New mutations in two Dutch patients with early infantile galactosialidosis
    J Groener
    Department of Clinical Genetics, Leiden University Medical Center, Leiden, The Netherlands
    Mol Genet Metab 78:222-8. 2003
  6. doi Muscle adaptation to short-term fasting in healthy lean humans
    Maarten R Soeters
    Department of Endocrinology and Metabolism, Academic Medical Center, Amsterdam, The Netherlands
    J Clin Endocrinol Metab 93:2900-3. 2008
  7. doi Dual-action lipophilic iminosugar improves glycemic control in obese rodents by reduction of visceral glycosphingolipids and buffering of carbohydrate assimilation
    Tom Wennekes
    Gorlaeus Laboratories, Leiden Institute of Chemistry, Leiden University, Leiden, The Netherlands
    J Med Chem 53:689-98. 2010
  8. doi Biomarkers for lysosomal storage disorders: identification and application as exemplified by chitotriosidase in Gaucher disease
    Johannes M Aerts
    Clinical Proteomics Facility, Academic Medical Center, Amsterdam, The Netherlands
    Acta Paediatr Suppl 97:7-14. 2008
  9. pmc Elevated globotriaosylsphingosine is a hallmark of Fabry disease
    Johannes M Aerts
    Amsterdam Lysosome Center, Departments of Medical Biochemistry, Internal Medicine, and Paediatrics, Academic Medical Center, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands
    Proc Natl Acad Sci U S A 105:2812-7. 2008
  10. ncbi Gender-related differences in the metabolic response to fasting
    Maarten R Soeters
    Department of Endocrinology and Metabolism, Laboratory of Endocrinology, Academic Medical Center, PO Box 22660, 1100 DD Amsterdam, The Netherlands
    J Clin Endocrinol Metab 92:3646-52. 2007

Collaborators

Detail Information

Publications21

  1. pmc Consequences of a global enzyme shortage of agalsidase beta in adult Dutch Fabry patients
    Bouwien E Smid
    Department of Internal Medicine, Division of Endocrinology and Metabolism, Academic Medical Centre, PO Box 22660, 1100 DD, Amsterdam, The Netherlands
    Orphanet J Rare Dis 6:69. 2011
    ..We determined the influence of the shortage on clinical event incidence and the most sensitive biochemical marker (lysoGb3) in Dutch Fabry patients...
  2. doi Ceramide in lipid emulsions used in parenteral nutrition: an innocent bystander?
    Johanna E Groener
    Department of Medical Biochemistry, Academic Medical Center, Amsterdam, The Netherlands
    JPEN J Parenter Enteral Nutr 35:270-1. 2011
    ..Further research is needed to determine whether this potential harmful bioactive compound is involved in parenteral nutrition-associated liver disease...
  3. ncbi Plasma glucosylceramide and ceramide in type 1 Gaucher disease patients: correlations with disease severity and response to therapeutic intervention
    J E M Groener
    Department of Medical Biochemistry, University of Amsterdam, Academic Medical Center, Amsterdam, The Netherlands
    Biochim Biophys Acta 1781:72-8. 2008
    ..In conclusion, plasma GlcCer concentration and GlcCer/Cer ratio is of value to monitor Gaucher disease manifestation and response to therapeutic intervention...
  4. ncbi HPLC for simultaneous quantification of total ceramide, glucosylceramide, and ceramide trihexoside concentrations in plasma
    Johanna E M Groener
    Department of Medical Biochemistry, University of Amsterdam, Academic Medical Center, Amsterdam, The Netherlands
    Clin Chem 53:742-7. 2007
    ..We developed an HPLC method for simultaneous quantification of total plasma concentrations of Cer, glucosylceramide (GlcCer), and ceramide trihexoside (CTH)...
  5. ncbi New mutations in two Dutch patients with early infantile galactosialidosis
    J Groener
    Department of Clinical Genetics, Leiden University Medical Center, Leiden, The Netherlands
    Mol Genet Metab 78:222-8. 2003
    ..Case 2 was homozygous for the same C899 insertion found in case 1...
  6. doi Muscle adaptation to short-term fasting in healthy lean humans
    Maarten R Soeters
    Department of Endocrinology and Metabolism, Academic Medical Center, Amsterdam, The Netherlands
    J Clin Endocrinol Metab 93:2900-3. 2008
    ..Intramyocellular sphingolipids (i.e. ceramide) have been suggested to induce insulin resistance by interfering with the insulin signaling cascade in obesity...
  7. doi Dual-action lipophilic iminosugar improves glycemic control in obese rodents by reduction of visceral glycosphingolipids and buffering of carbohydrate assimilation
    Tom Wennekes
    Gorlaeus Laboratories, Leiden Institute of Chemistry, Leiden University, Leiden, The Netherlands
    J Med Chem 53:689-98. 2010
    ..We conclude that the combination of reduction of glycosphingolipids in tissue and buffering of carbohydrate assimilation by 2 produces a superior glucose homeostasis...
  8. doi Biomarkers for lysosomal storage disorders: identification and application as exemplified by chitotriosidase in Gaucher disease
    Johannes M Aerts
    Clinical Proteomics Facility, Academic Medical Center, Amsterdam, The Netherlands
    Acta Paediatr Suppl 97:7-14. 2008
    ..Recent developments in biomarker discovery by proteomics are described and the future for biomarkers of LSDs is discussed...
  9. pmc Elevated globotriaosylsphingosine is a hallmark of Fabry disease
    Johannes M Aerts
    Amsterdam Lysosome Center, Departments of Medical Biochemistry, Internal Medicine, and Paediatrics, Academic Medical Center, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands
    Proc Natl Acad Sci U S A 105:2812-7. 2008
    ..Our findings suggest that measurement of circulating globotriaosylsphingosine will be useful to monitor Fabry disease and may contribute to a better understanding of the disorder...
  10. ncbi Gender-related differences in the metabolic response to fasting
    Maarten R Soeters
    Department of Endocrinology and Metabolism, Laboratory of Endocrinology, Academic Medical Center, PO Box 22660, 1100 DD Amsterdam, The Netherlands
    J Clin Endocrinol Metab 92:3646-52. 2007
    ..During fasting, women have lower plasma glucose levels than men despite higher plasma FFA, suggesting protection from FFA-induced insulin resistance...
  11. ncbi Chronic treatment with pioglitazone does not protect obese patients with diabetes mellitus type II from free fatty acid-induced insulin resistance
    Mireille J Serlie
    Department of Endocrinology and Metabolism, Academic Medical Center, F5 169, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
    J Clin Endocrinol Metab 92:166-71. 2007
    ..Thiazolidinediones increase peripheral insulin sensitivity and decrease plasma free fatty acids (FFA). However, their exact mechanism of action has not been fully elucidated...
  12. ncbi Type I Gaucher disease, a glycosphingolipid storage disorder, is associated with insulin resistance
    Mirjam Langeveld
    Academic Medical Center, Department of Endocrinology and Metabolism F4 247, Meibergdreef 9, 1105AZ Amsterdam, The Netherlands
    J Clin Endocrinol Metab 93:845-51. 2008
    ..Secondary to this defect, GM3 concentrations, for which glucosylceramide is the precursor, in plasma and several cell types are elevated...
  13. ncbi Substrate reduction therapy of glycosphingolipid storage disorders
    Johannes M F G Aerts
    Department of Medical Biochemistry, Academic Medical Center, University of Amsterdam, Meibergdreef 15, 1105 AZ, Amsterdam, The Netherlands
    J Inherit Metab Dis 29:449-56. 2006
    ....
  14. ncbi CCL18: a urinary marker of Gaucher cell burden in Gaucher patients
    Rolf G Boot
    Department of Medical Biochemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
    J Inherit Metab Dis 29:564-71. 2006
    ..Urinary chitotriosidase appears rather to be a reflection of renal pathology...
  15. ncbi Low frequency maintenance therapy with imiglucerase in adult type I Gaucher disease: a prospective randomized controlled trial
    Maaike de Fost
    Department of Endocrinology and Metabolism, F4 279, University of Amsterdam, Academic Medical Center P O Box 22700, 1100 DD Amsterdam, The Netherlands
    Haematologica 92:215-21. 2007
    ..In order to reduce the burden of the intravenously administered enzyme, a low frequency of administration was prospectively studied in patients with stable and minor disease following ERT...
  16. ncbi Transglycosidase activity of chitotriosidase: improved enzymatic assay for the human macrophage chitinase
    Begoña Aguilera
    Department of Biochemistry, Academic Medical Center, University of Amsterdam, Meibergdreef 15, 1105 AZ Amsterdam, The Netherlands
    J Biol Chem 278:40911-6. 2003
    ..The novel convenient chitotriosidase enzyme assay should facilitate the accurate monitoring of Gaucher disease patients receiving costly enzyme replacement therapy...
  17. ncbi Treatment of Fabry disease with different dosing regimens of agalsidase: effects on antibody formation and GL-3
    Anouk C Vedder
    Department of Internal Medicine Endocrinology and Metabolism, Academic Medical Center, F4 224, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
    Mol Genet Metab 94:319-25. 2008
    ..Infusion of a dose of 1.0mg/kg results in a more robust decline in GL-3, less impact, if any of antibodies, stable renal function and reduction of LVMass...
  18. doi Prominent increase in plasma ganglioside GM3 is associated with clinical manifestations of type I Gaucher disease
    Karen Ghauharali-van der Vlugt
    Department of Medical Biochemistry and Internal Medicine, Academic Medical Center, University of Amsterdam, The Netherlands
    Clin Chim Acta 389:109-13. 2008
    ..The goal of the current study was to establish whether GM3 is elevated in plasma of type I Gaucher disease patients, and is related to disease manifestations...
  19. doi Detection of chitinase activity by 2-aminobenzoic acid labeling of chito-oligosaccharides
    Karen Ghauharali-van der Vlugt
    Department of Medical Biochemistry, Academic Medical Center, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands
    Anal Biochem 384:191-3. 2009
    ..Comparison with existing UV-based assays, shows that the novel assay offers the same advantages yet allows detection of chito-oligosaccharides in the low picomolar range...
  20. ncbi Superior effects of high-dose enzyme replacement therapy in type 1 Gaucher disease on bone marrow involvement and chitotriosidase levels: a 2-center retrospective analysis
    Maaike de Fost
    Department of Internal Medicine, Endocrinology and Metabolism, F4 279, University of Amsterdam, Academic Medical Center, 1100 DD Amsterdam, The Netherlands
    Blood 108:830-5. 2006
    ....
  21. doi Spontaneous regression of disease manifestations can occur in type 1 Gaucher disease; results of a retrospective cohort study
    Jooske M F Boomsma
    Department of Internal Medicine, Academic Medical Center, Amsterdam, The Netherlands
    Blood Cells Mol Dis 44:181-7. 2010
    ..None of the parameters was predictive of progression or regression of disease. In conclusion, GD in adults can, in some cases, regress spontaneously. No parameters for accurately predicting future disease course exist...