Results 51 to 60 of about 151,232 (337)

The epitope of the antibody used in the REAADS VWF activity assay is quaternary

Thrombosis Journal
The REAADS VWF activity assay is often assumed to be specific for the A1 domain, the portion of VWF that binds platelet GPIbα. We tested this assay on the A1A2A3 region of VWF with each domain expressed independently of one another and together in ...
Alexander Tischer, Laurie Moon-Tasson, Matthew Auton   +2 more
doaj   +1 more source

Evaluation of the Utility of von Willebrand Factor Propeptide in the Differential Diagnosis of von Willebrand Disease and Acquired von Willebrand Syndrome

Seminars in Thrombosis and Hemostasis, 2018
An increased von Willebrand factor propeptide (VWFpp) to VWF antigen (VWF:Ag) ratio (VWFpp/VWF:Ag) indicates an enhanced clearance of VWF. This finding has been described in von Willebrand disease (VWD) and in acquired von Willebrand syndrome (AVWS).
F. Stufano, M. Boscarino, P. Bucciarelli, L. Baronciani, A. Maino, G. Cozzi, F. Peyvandi   +6 more
semanticscholar   +1 more source

A Biomarker‐Driven Ovary–Endometrium Organ‐on‐a‐Chip Mimicking 3D Multicellular Complexity and Menstrual Cyclicity for Predicting Reproductive Toxicity

Advanced Science, EarlyView.
We present a dual‐organ, biomarker‐integrated ovaryendometrium organ‐on‐a‐chip that recapitulates 3D tissue complexity, menstrual cycle dynamics, and hormonal crosstalk. This platform enables real‐time, cell‐typespecific fluorescent readouts of reproductive toxicity using ANGPTL4 and SERPINB2 as early‐response reporters.
Soo‐Rim Kim, Eun‐kyung Min, Choon‐Mi Lee, Chan Hum Park, Byung‐Chul Oh, YunJae Jung, In‐Sun Hong   +6 more
wiley   +1 more source

Modulating Integrin and Growth Factor Signaling With Peptides: Strategies to Synergistically Enhance Bone Tissue Regeneration

Advanced Science, EarlyView.
It has been demonstrated that, in the bone extracellular matrix (ECM), integrins and growth factor receptors (GFRs) engage in synergistic signaling to guide bone healing and regeneration. This review provides a comprehensive overview of current strategies using ECM‐derived peptides to recreate the cellular microenvironment and harness synergistic ...
Lluís Oliver‐Cervelló, Elisabetta Ada Cavalcanti‐Adam, Carlos Mas‐Moruno   +2 more
wiley   +1 more source

High and long‐term von Willebrand factor expression after Sleeping Beauty transposon‐mediated gene therapy in a mouse model of severe von Willebrand disease

Journal of Thrombosis and Haemostasis, 2018
Essentials von Willebrand disease (VWD) is the most common inherited bleeding disorder. Gene therapy for VWD offers long‐term therapy for VWD patients. Transposons efficiently integrate the large von Willebrand factor (VWF) cDNA in mice.
I. Portier, K. Vanhoorelbeke, S. Verhenne, I. Pareyn, N. Vandeputte, H. Deckmyn, D. Goldenberg, H. B. Samal, M. Singh, Z. Ivics, Z. Izsvák, S. D. De Meyer   +11 more
semanticscholar   +1 more source

Proteomic Analysis of Golden Sputum Reveals Pulmonary Complement Activation During Acute Chest Syndrome in Children With Sickle Cell Disease

American Journal of Hematology, EarlyView.
ABSTRACT Acute chest syndrome (ACS) is one of the most common severe complications of sickle cell disease (SCD). In recent years, a major role of inflammation and innate immunity has been evidenced, but ACS pathophysiology remains incompletely understood, and therapeutic options are limited.
Slimane Allali, Nabiha Sbeih, Rachel Rignault‐Bricard, Johanna Bruce, Morgane Le Gall, Claire Heilbronner, Noemie de Cacqueray, Sofia Angyalosy, Melissa Taylor, Joséphine Brice, Mariane de Montalembert, Martina Bevacqua, Emilie‐Fleur Gautier, Olivier Hermine, Thiago Trovati Maciel   +14 more
wiley   +1 more source

High-throughput molecular diagnosis of von Willebrand disease by next generation sequencing methods

Haematologica, 2012
Genetic analysis of von Willebrand disease by von Willebrand factor gene sequencing has not yet become routine practice. Nevertheless, the prospects for molecular diagnosis have changed dramatically in recent years with the unveiling of next-generation ...
Irene Corrales, Susana Catarino, Júlia Ayats, David Arteta, Carmen Altisent, Rafael Parra, Francisco Vidal   +6 more
doaj   +1 more source

Non‐RASopathy Genetic Syndromes Identified as the Molecular Cause of Disease in Patients Previously Diagnosed With Noonan Syndrome

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Noonan Syndrome (NS) is a clinically and genetically heterogeneous condition characterized by typical facial dysmorphisms, short stature, congenital heart defects, and developmental delays. While variants in genes such as PTPN11, SOS1, and RAF1 account for most genetically confirmed cases, diagnosis is challenging due to phenotypic overlap ...
Gabriela Jeesoo Kim, Alexsandra Christianne Malaquias, Debora Romeo Bertola, Raissa Carneiro Rezende, Laurana De Polli Cellin, Lucas Vieira Lacerda Pires, Ana Maria Santillan‐Vasconez, Antônio Marcondes Lerario, Renata da Cunha Scalco, Alexander Augusto de Lima Jorge   +9 more
wiley   +1 more source

Correlation of intracardiac hemodynamics indicators with von Willebrand factor – Marker of endothelial dysfunction in patients with coronary artery disease combined with coronavirus disease (COVID-19) [PDF]

Българска кардиология
The research relevance is predefined by the need to establish the relationship and identify new markers and potential contributions to clinical practice and scientific progress.
V. Netiazhenko, S. Mostovyi, O. Safonova
doaj   +3 more sources

Long‐Read Genome Sequencing Establishes Biallelic Pathogenic Variants in DNM1 With Distinct Functional Effects as the Cause of Early Infantile Developmental and Epileptic Encephalopathy

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Heterozygous de novo and inherited biallelic pathogenic variants in DNM1 have been reported in association with autosomal dominant (AD) and autosomal recessive (AR) developmental and epileptic encephalopathy, respectively, due to aberrant dynamin function or expression, with each inheritance pattern associated with a different mechanism of ...
Andy Drackley, Merlene Peter, Heba H. Akbari, Jelena Ivanisevic, Alexander Ing, Kelly Regan‐Fendt, Patrick McMullen, Kai Lee Yap   +7 more
wiley   +1 more source