List of works - Johannes M F G Aerts

A single histidine residue modulates enzymatic activity in acidic mammalian chitinase

scientific article

Abnormal nonstoring capillary endothelium: a novel feature of Gaucher disease. Ultrastructural study of dermal capillaries

scientific article

Assessing the statistical validity of proteomics based biomarkers

scientific article published on 27 April 2007

Assessment of partially deoxygenated deoxynojirimycin derivatives as glucosylceramide synthase inhibitors

scientific article published on 7 April 2011

Biomarkers in the diagnosis of lysosomal storage disorders: proteins, lipids, and inhibodies

scientific article

Cell surface associated glycohydrolases in normal and Gaucher disease fibroblasts

scientific article

Common G102S polymorphism in chitotriosidase differentially affects activity towards 4-methylumbelliferyl substrates

scientific article

Consequences of a global enzyme shortage of agalsidase beta in adult Dutch Fabry patients

scientific article published on 31 October 2011

Convection perfusion of glucocerebrosidase for neuronopathic Gaucher's disease

scientific article published in April 2005

Correction of liver steatosis by a hydrophobic iminosugar modulating glycosphingolipids metabolism

scientific article

Distinguishing the differences in beta-glycosylceramidase folds, dynamics, and actions informs therapeutic uses

article

Dual-action lipophilic iminosugar improves glycemic control in obese rodents by reduction of visceral glycosphingolipids and buffering of carbohydrate assimilation

scientific article published on January 2010

Effects of switching from a reduced dose imiglucerase to velaglucerase in type 1 Gaucher disease: clinical and biochemical outcomes

scientific article published on 6 July 2012

Enzyme therapy for Fabry disease: neutralizing antibodies toward agalsidase alpha and beta

scientific article published in October 2004

Enzyme therapy for the treatment of type 1 Gaucher disease: clinical outcomes and dose - response relationships

scientific article

Exploring functional cyclophellitol analogues as human retaining beta-glucosidase inhibitors

scientific article published on 26 August 2014

Fasting-induced myocardial lipid accumulation in long-chain acyl-CoA dehydrogenase knockout mice is accompanied by impaired left ventricular function

scientific article (publication date: September 2011)

Force majeure: therapeutic measures in response to restricted supply of imiglucerase (Cerezyme) for patients with Gaucher disease

scientific article published on 4 October 2009

Gaucher cells demonstrate a distinct macrophage phenotype and resemble alternatively activated macrophages

scientific article published in September 2004

Gaucher disease: a model disorder for biomarker discovery

scientific article published on August 2009

Glucocerebrosidase: Functions in and Beyond the Lysosome

scientific article published on 09 March 2020

Glucocerebrosidases catalyze a transgalactosylation reaction that yields a newly identified brain sterol metabolite, galactosylated cholesterol

scientific article published on 06 March 2020

Glucosylated cholesterol in skin: synthetic role of extracellular glucocerebrosidase

scientific article published on 15 September 2020

Glycosphingolipids and insulin resistance.

scientific article published on 20 March 2009

Hepatic glycosphingolipid deficiency and liver function in mice

scientific article published in May 2010

Human acidic mammalian chitinase erroneously known as eosinophil chemotactic cytokine is not the ortholog of mouse YM1

scientific article published on 01 August 2005

Iminosugar-based inhibitors of glucosylceramide synthase increase brain glycosphingolipids and survival in a mouse model of Sandhoff disease

scientific article

Inhibition of glycosphingolipid synthesis induces a profound reduction of plasma cholesterol and inhibits atherosclerosis development in APOE*3 Leiden and low-density lipoprotein receptor-/- mice

scientific article published on 18 February 2010

Investigations on therapeutic glucocerebrosidases through paired detection with fluorescent activity-based probes

scientific article

Limitations of drug registries to evaluate orphan medicinal products for the treatment of lysosomal storage disorders ⬇️

scientific article

Linking Smokers' Susceptibility to Tuberculosis with Lysosomal Storage Disorders.

scientific article published on April 2016

Long-term effect of antibodies against infused alpha-galactosidase A in Fabry disease on plasma and urinary (lyso)Gb3 reduction and treatment outcome

scientific article

Low-dose versus high-dose therapy for Gaucher disease: Goals and markers

scientific article published on 01 January 2007

Manno-epi-cyclophellitols Enable Activity-Based Protein Profiling of Human α-Mannosidases and Discovery of New Golgi Mannosidase II Inhibitors

scientific article published on 30 June 2020

Nanomolar affinity, iminosugar-based chemical probes for specific labeling of lysosomal glucocerebrosidase

scientific article published on 31 October 2009

Novel defect in phosphatidylinositol 4-kinase type 2-alpha (PI4K2A) at the membrane-enzyme interface is associated with metabolic cutis laxa

scientific article published on 17 May 2020

Phenotype diversity in type 1 Gaucher disease: discovering the genetic basis of Gaucher disease/hematologic malignancy phenotype by individual genome analysis

scientific article

Plant Glycosides and Glycosidases: A Treasure-Trove for Therapeutics

scientific article published on 07 April 2020

Recombinant enzyme therapy for Fabry disease: absence of editing of human alpha-galactosidase A mRNA.

scientific article

Reducing GBA2 Activity Ameliorates Neuropathology in Niemann-Pick Type C Mice

scientific article (publication date: 2015)

Reduction of glycosphingolipid biosynthesis stimulates biliary lipid secretion in mice

scientific article

Selective Cross-Metathesis of C-Allyl-glycosides

Selective Cross‐Metathesis ofC‐Allyl‐Glycosides

Small fiber neuropathy in Fabry disease

scientific article published on 24 March 2012

Spontaneous regression of disease manifestations can occur in type 1 Gaucher disease; results of a retrospective cohort study

scientific article

Structure of human chitotriosidase. Implications for specific inhibitor design and function of mammalian chitinase-like lectins

scientific article

Synthesis and Evaluation of Hybrid Structures Composed of Two Glucosylceramide Synthase Inhibitors

scientific article published on 23 October 2015

TLR- and NOD2-dependent regulation of human phagocyte-specific chitotriosidase

scientific article published on 30 October 2007

The cytosolic β-glucosidase GBA3 does not influence type 1 Gaucher disease manifestation

scientific article

Treatment of Fabry disease: outcome of a comparative trial with agalsidase alfa or beta at a dose of 0.2 mg/kg

scientific article published in 2007

Type I Gaucher disease, a glycosphingolipid storage disorder, is associated with insulin resistance

scientific article

List of works by Johannes M F G Aerts

A single histidine residue modulates enzymatic activity in acidic mammalian chitinase

Abnormal nonstoring capillary endothelium: a novel feature of Gaucher disease. Ultrastructural study of dermal capillaries

Assessing the statistical validity of proteomics based biomarkers

Assessment of partially deoxygenated deoxynojirimycin derivatives as glucosylceramide synthase inhibitors

Biomarkers in the diagnosis of lysosomal storage disorders: proteins, lipids, and inhibodies

Cell surface associated glycohydrolases in normal and Gaucher disease fibroblasts

Common G102S polymorphism in chitotriosidase differentially affects activity towards 4-methylumbelliferyl substrates

Consequences of a global enzyme shortage of agalsidase beta in adult Dutch Fabry patients

Convection perfusion of glucocerebrosidase for neuronopathic Gaucher's disease

Correction of liver steatosis by a hydrophobic iminosugar modulating glycosphingolipids metabolism

Distinguishing the differences in beta-glycosylceramidase folds, dynamics, and actions informs therapeutic uses

Dual-action lipophilic iminosugar improves glycemic control in obese rodents by reduction of visceral glycosphingolipids and buffering of carbohydrate assimilation

Effects of switching from a reduced dose imiglucerase to velaglucerase in type 1 Gaucher disease: clinical and biochemical outcomes

Enzyme therapy for Fabry disease: neutralizing antibodies toward agalsidase alpha and beta

Enzyme therapy for the treatment of type 1 Gaucher disease: clinical outcomes and dose - response relationships

Exploring functional cyclophellitol analogues as human retaining beta-glucosidase inhibitors

Fasting-induced myocardial lipid accumulation in long-chain acyl-CoA dehydrogenase knockout mice is accompanied by impaired left ventricular function

Force majeure: therapeutic measures in response to restricted supply of imiglucerase (Cerezyme) for patients with Gaucher disease

Gaucher cells demonstrate a distinct macrophage phenotype and resemble alternatively activated macrophages

Gaucher disease: a model disorder for biomarker discovery

Glucocerebrosidase: Functions in and Beyond the Lysosome

Glucocerebrosidases catalyze a transgalactosylation reaction that yields a newly identified brain sterol metabolite, galactosylated cholesterol

Glucosylated cholesterol in skin: synthetic role of extracellular glucocerebrosidase

Glycosphingolipids and insulin resistance.

Hepatic glycosphingolipid deficiency and liver function in mice

Human acidic mammalian chitinase erroneously known as eosinophil chemotactic cytokine is not the ortholog of mouse YM1

Iminosugar-based inhibitors of glucosylceramide synthase increase brain glycosphingolipids and survival in a mouse model of Sandhoff disease

Inhibition of glycosphingolipid synthesis induces a profound reduction of plasma cholesterol and inhibits atherosclerosis development in APOE*3 Leiden and low-density lipoprotein receptor-/- mice

Investigations on therapeutic glucocerebrosidases through paired detection with fluorescent activity-based probes

Limitations of drug registries to evaluate orphan medicinal products for the treatment of lysosomal storage disorders ⬇️

Linking Smokers' Susceptibility to Tuberculosis with Lysosomal Storage Disorders.

Long-term effect of antibodies against infused alpha-galactosidase A in Fabry disease on plasma and urinary (lyso)Gb3 reduction and treatment outcome

Low-dose versus high-dose therapy for Gaucher disease: Goals and markers

Manno-epi-cyclophellitols Enable Activity-Based Protein Profiling of Human α-Mannosidases and Discovery of New Golgi Mannosidase II Inhibitors

Nanomolar affinity, iminosugar-based chemical probes for specific labeling of lysosomal glucocerebrosidase

Novel defect in phosphatidylinositol 4-kinase type 2-alpha (PI4K2A) at the membrane-enzyme interface is associated with metabolic cutis laxa

Phenotype diversity in type 1 Gaucher disease: discovering the genetic basis of Gaucher disease/hematologic malignancy phenotype by individual genome analysis

Plant Glycosides and Glycosidases: A Treasure-Trove for Therapeutics

Recombinant enzyme therapy for Fabry disease: absence of editing of human alpha-galactosidase A mRNA.

Reducing GBA2 Activity Ameliorates Neuropathology in Niemann-Pick Type C Mice

Reduction of glycosphingolipid biosynthesis stimulates biliary lipid secretion in mice

Selective Cross-Metathesis of C-Allyl-glycosides

Selective Cross‐Metathesis ofC‐Allyl‐Glycosides

Small fiber neuropathy in Fabry disease

Spontaneous regression of disease manifestations can occur in type 1 Gaucher disease; results of a retrospective cohort study

Structure of human chitotriosidase. Implications for specific inhibitor design and function of mammalian chitinase-like lectins

Synthesis and Evaluation of Hybrid Structures Composed of Two Glucosylceramide Synthase Inhibitors

TLR- and NOD2-dependent regulation of human phagocyte-specific chitotriosidase

The cytosolic β-glucosidase GBA3 does not influence type 1 Gaucher disease manifestation

Treatment of Fabry disease: outcome of a comparative trial with agalsidase alfa or beta at a dose of 0.2 mg/kg

Type I Gaucher disease, a glycosphingolipid storage disorder, is associated with insulin resistance