Human Alpha Galactosidases Transiently Produced in Nicotiana benthamiana Leaves: New Insights in Substrate Specificities with Relevance for Fabry Disease

Chemoenzymatic Synthesis of a Phosphorylated Glycoprotein

(2016) Pragya Priyanka et al.   ANGEWANDTE CHEMIE-INTERNATIONAL EDITION

Lysosomal glycosphingolipid catabolism by acid ceramidase: formation of glycosphingoid bases during deficiency of glycosidases

(2016) Maria J. Ferraz et al.   FEBS LETTERS

Lyso-glycosphingolipid abnormalities in different murine models of lysosomal storage disorders

(2016) Maria J. Ferraz et al.   MOLECULAR GENETICS AND METABOLISM

Lyso-Gb3 activates Notch1 in human podocytes

(2015) Maria D. Sanchez-Niño et al.   HUMAN MOLECULAR GENETICS

Mannose receptor-mediated delivery of moss-made α-galactosidase A efficiently corrects enzyme deficiency in Fabry mice

(2015) Jin-Song Shen et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Characterization of a chemically modified plant cell culture expressed human α-Galactosidase-A enzyme for treatment of Fabry disease

(2015) Tali Kizhner et al.   MOLECULAR GENETICS AND METABOLISM

The Fabry disease-associated lipid Lyso-Gb3 enhances voltage-gated calcium currents in sensory neurons and causes pain

(2015) L. Choi et al.   NEUROSCIENCE LETTERS

The quality of economic evaluations of ultra-orphan drugs in Europe – a systematic review

(2015) Y. Schuller et al.   Orphanet Journal of Rare Diseases

The N-glycan on Asn54 affects the atypical N-glycan composition of plant-produced interleukin-22, but does not influence its activity

(2015) Ruud H. P. Wilbers et al.   PLANT BIOTECHNOLOGY JOURNAL

Moss-made pharmaceuticals: from bench to bedside

(2015) Ralf Reski et al.   PLANT BIOTECHNOLOGY JOURNAL

Oral delivery of Acid Alpha Glucosidase epitopes expressed in plant chloroplasts suppresses antibody formation in treatment of Pompe mice

(2015) Jin Su et al.   PLANT BIOTECHNOLOGY JOURNAL

Potent and Selective Activity-Based Probes for GH27 Human Retaining α-Galactosidases

(2014) Lianne I. Willems et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Monomeric IgA can be producedin plantaas efficient as IgG, yet receives differentN-glycans

(2014) Lotte B. Westerhof et al.   PLANT BIOTECHNOLOGY JOURNAL

Efficient Agroinfiltration of Plants for High-level Transient Expression of Recombinant Proteins

(2013) Kahlin Leuzinger et al.   Jove-Journal of Visualized Experiments

Glyco-engineering in plants to produce human-like N-glycan structures

(2012) Alexandra Castilho et al.   Biotechnology Journal

Taliglucerase alfa leads to favorable bone marrow responses in patients with type I Gaucher disease

(2012) L. van Dussen et al.   BLOOD CELLS MOLECULES AND DISEASES

Quantification of Globotriaosylsphingosine in Plasma and Urine of Fabry Patients by Stable Isotope Ultraperformance Liquid Chromatography-Tandem Mass Spectrometry

(2012) H. Gold et al.   CLINICAL CHEMISTRY

3D Domain Swapping Causes Extensive Multimerisation of Human Interleukin-10 When Expressed In Planta

(2012) Lotte B. Westerhof et al.   PLoS One

Long-Term Effect of Antibodies against Infused Alpha-Galactosidase A in Fabry Disease on Plasma and Urinary (lyso)Gb3 Reduction and Treatment Outcome

(2012) Saskia M. Rombach et al.   PLoS One

Algae as Protein Factories: Expression of a Human Antibody and the Respective Antigen in the Diatom Phaeodactylum tricornutum

(2011) Franziska Hempel et al.   PLoS One

Reduction of elevated plasma globotriaosylsphingosine in patients with classic Fabry disease following enzyme replacement therapy

(2010) Mariëlle J. van Breemen et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Interconversion of the Specificities of Human Lysosomal Enzymes Associated with Fabry and Schindler Diseases

(2010) Ivan B. Tomasic et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Ultrasensitive in situ visualization of active glucocerebrosidase molecules

(2010) Martin D Witte et al.   Nature Chemical Biology

Use of a Modified α-N-Acetylgalactosaminidase in the Development of Enzyme Replacement Therapy for Fabry Disease

(2009) Youichi Tajima et al.   AMERICAN JOURNAL OF HUMAN GENETICS

High Incidence of the Cardiac Variant of Fabry Disease Revealed by Newborn Screening in the Taiwan Chinese Population

(2009) Hsiang-Yu Lin et al.   Circulation-Cardiovascular Genetics

Catalytic Mechanism of Human α-Galactosidase

(2009) Abigail I. Guce et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The 1.9 Å Structure of Human α-N-Acetylgalactosaminidase: The Molecular Basis of Schindler and Kanzaki Diseases

(2009) Nathaniel E. Clark et al.   JOURNAL OF MOLECULAR BIOLOGY

Elevated globotriaosylsphingosine is a hallmark of Fabry disease

(2008) J. M. Aerts et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA