Results 51 to 60 of about 30,590 (168)

Occurrence of Infusion Associated Reactions and Antidrug Antibodies in Enzyme Replacement Therapy for Fabry Disease and the Effect of Preventive Measures

Journal of Inherited Metabolic Disease, Volume 49, Issue 5, September 2026.
Occurrence of infusion associated reactions and anti‐drug antibodies in enzyme replacement therapy for Fabry disease and the effect of preventive measures. ABSTRACT Male patients with the classical phenotype of Fabry disease (FD) are at risk of developing inhibiting antidrug antibodies (iADAs) against recombinant α‐galactosidase‐A, administered in the ...
Maud Janssens, Sanne J. van der Veen, Laura van Dussen, André B. P. van Kuilenburg, Marion M. M. G. Brands, Mirjam Langeveld   +5 more
wiley   +1 more source

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

Frontiers in Plant Science, 2017
Deficiency of α-galactosidase A (α-GAL) causes Fabry disease (FD), an X-linked storage disease of the glycosphingolipid globtriaosylcerammide (Gb3) in lysosomes of various cells and elevated plasma globotriaosylsphingosine (Lyso-Gb3) toxic for podocytes ...
Kassiani Kytidou, Thomas J. M. Beenakker, Lotte B. Westerhof, Cornelis H. Hokke, Geri F. Moolenaar, Nora Goosen, Mina Mirzaian, Maria J. Ferraz, Mark de Geus, Wouter W. Kallemeijn, Herman S. Overkleeft, Rolf G. Boot, Arjen Schots, Dirk Bosch, Johannes M. F. G. Aerts   +14 more
doaj   +1 more source

Human Cytomegalovirus Genetic Diversity, Clinical Relevance, and Emerging Insights

Reviews in Medical Virology, Volume 36, Issue 5, September 2026.
ABSTRACT Human cytomegalovirus (HCMV) is a globally prevalent virus that poses a significant public health concern, especially for newborns and immunocompromised patients, causing a wide range of infections. Clinical outcomes associated with HCMV infection include congenital disease, graft rejection in transplant recipients, and life‐threatening ...
Hajar Y. AlQahtani, Fadilah Sfouq Aleanizy, Fulwah Yahya Alqahtani   +2 more
wiley   +1 more source

Unveiling Van Der Waals Forces and Associated Hamaker Constants Across the Nanomaterial Landscape With Advanced Atomic Force Microscopy

Small Methods, Volume 10, Issue 18, 23 September 2026.
Van der Waals interactions are reviewed through the lens of advanced atomic force microscopy, highlighting recent progress in nanoscale quantification of Hamaker constants. Emerging methodologies enable high‐resolution mapping of intermolecular forces in 2D materials and biological systems, bridging experimental measurements with quantum‐informed ...
Amir Farokh Payam, Horacio V. Guzman, Alexandre Tkatchenko   +2 more
wiley   +1 more source

Human Gb3/CD77 synthase: a glycosyltransferase at the crossroads of immunohematology, toxicology, and cancer research

Cellular & Molecular Biology Letters
Human Gb3/CD77 synthase (α1,4-galactosyltransferase, P1/Pk synthase, UDP-galactose: β-d-galactosyl-β1-R 4-α-d-galactosyltransferase, EC 2.4.1.228) forms Galα1 → 4Gal structures on glycosphingolipids and glycoproteins.
Katarzyna Szymczak-Kulus, Marcin Czerwinski, Radoslaw Kaczmarek   +2 more
doaj   +1 more source

Functional Specialization of Clathrin‐Independent Endocytic Routes: Mechanisms and Cellular Roles

Traffic, Volume 27, Issue 3, September 2026.
Distinct clathrin‐independent endocytic (CIE) routes support specialized cellular functions across different contexts. Created with Biorender.com. ABSTRACT Clathrin‐independent endocytosis (CIE) comprises a diverse repertoire of internalization mechanisms that operate in parallel with clathrin‐mediated endocytosis.
Andrea Francesco Benvenuto, Amir Fardin, Matteo Balestra, Pier Paolo Di Fiore, Chiara Tordonato, Sara Sigismund   +5 more
wiley   +1 more source

Fatty acid-dependent globotriaosyl ceramide receptor function in detergent resistant model membranes

Journal of Lipid Research, 2009
Glycosphingolipid (GSL) fatty acid strictly regulates verotoxin 1 (VT1) and the HIV adhesin, gp120 binding to globotriaosyl ceramide within Gb3/cholesterol detergent resistant membrane (DRM) vesicle constructs and in Gb3 water-air interface monolayers in
Radhia Mahfoud, Adam Manis, Clifford A. Lingwood   +2 more
doaj   +1 more source

Massive accumulation of globotriaosylceramide in various tissues from a Fabry patient with a high antibody titer against alpha-galactosidase A after 6 years of enzyme replacement therapy

Molecular Genetics and Metabolism Reports, 2020
Fabry disease is an X-linked metabolic disorder due to a pathogenic mutation of the GLA gene. The accumulation of globotriaosylceramide (Gb3) damages multiple organs, including the heart, kidney and nervous system, especially in classical type Fabry ...
Kenichi Hongo, Toru Harada, Eiko Fukuro, Masahisa Kobayashi, Toya Ohashi, Yoshikatsu Eto   +5 more
doaj   +1 more source

Myocardial Lipid Metabolism Imbalance: The Pathological Core and Novel Diagnostic‐Therapeutic Directions of Cardiovascular Diseases

Journal of Biochemical and Molecular Toxicology, Volume 40, Issue 8, August 2026.
In cardiac cells, Plin5/AMPK regulate lipid homeostasis; excess CD36‐mediated uptake drives lipotoxicity, mitochondrial dysfunction, and CVDs (e.g., heart failure). Biomarkers (ApoB/ApoA‐1) and therapies (SGLT2 inhibitors) target this cascade. ABSTRACT Cardiac lipid metabolism is fundamental to myocardial energy homeostasis, with fatty acid oxidation ...
Peiyun Xie, Qun Zeng, Hongrui Wang, Meihua She   +3 more
wiley   +1 more source

Imbalanced Production of Reactive Oxygen Species and Mitochondrial Antioxidant SOD2 in Fabry Disease-Specific Human Induced Pluripotent Stem Cell-Differentiated Vascular Endothelial Cells

Cell Transplantation, 2017
Fabry disease (FD) is an X-linked inherited lysosomal storage disease caused by α-galactosidase A (GLA) deficiency. Progressive intracellular accumulation of globotriaosylceramide (Gb3) is considered to be pathogenically responsible for the phenotype ...
Wei-Lien Tseng, Shih-Jie Chou, Huai-Chih Chiang, Mong-Lien Wang, Chian-Shiu Chien, Kuan-Hsuan Chen, Hsin-Bang Leu, Chien-Ying Wang, Yuh-Lih Chang, Yung-Yang Liu, Yuh-Jyh Jong, Shinn-Zong Lin, Shih-Hwa Chiou, Shing-Jong Lin, Wen-Chung Yu   +14 more
doaj   +1 more source